PANCE Exam: Hematology Review Questions & Answers

ANEMIAS

Question: What is the most common cause of hyperviscosity syndrome?
Waldenstrom macroglobulinemia.

Question: In anemia, which way is the oxygen dissociation curve shifted?
The right (affinity of hemoglobin for oxygen is decreased).

Question: What are the factors contributing to the pathophysiology of anemia of chronic disease?
Decreased red cell life span (hyperactive reticuloendothelial system), hypoactive bone marrow, erythropoietin production inadequate for degree of anemia, and abnormal iron metabolism.

Question: What is the formula for calculating the proper volume of red cells to transfuse in an anemic patient?
Desired hemoglobin (g/dL) = observed hemoglobin (g/dL) × weight (kg) × 3.

Question: What should be considered in a patient who presents in a coma and with anemia and rouleaux formation in the peripheral blood smear?
Primary macroglobulinemia (hyperviscosity syndrome).

Question: A 20-year-old male patient presents with pancytopenia. On smear you note anemia associated with decreased reticulocytes and the morphology is unremarkable. Neutrophils and platelets are reduced in number and no immature or abnormal forms are seen. Bone marrow biopsy and aspirate is hypocellular. What is your most likely diagnosis?
Aplastic anemia.

Question: The primary cause of aplastic anemia is:
Unknown.

Aplastic anemia is characterized by two peaks in frequency of disease appearance. At what ages are these peaks?
Ages 15 to 25 years and 65 to 69 years.

Question: What diagnosis should be considered in a patient who presents with macrocytic anemia, macro-ovalocytes, and hypersegmented neutrophils on peripheral blood smear, along with a serum vitamin B12 level of < 100 pg/mL?
Vitamin B12 deficiency.

Question: A 36-year-old female patient presents to your clinic with anemia. In her history, you learn that she is a strict vegan (a strict vegetarian that avoids all animal products). What is at the top of your differential?
Vitamin B12 deficiency.

Question: What are the common signs and symptoms of Vitamin B12 deficiency?
- Decreased vibration and position sense is common early in the disease; later paresthesias, balance disorders, and impaired cerebral function develop
- Patients are usually pale and mildly icteric

Question: Treatment for pernicious anemia after initial replacement is provided by:
Intramuscular injections of 100 μg of vitamin B12 monthly for life.

Question: What diagnosis should be considered in a patient who presents with macrocytic anemia, macro-ovalocytes, and hypersegmented neutrophils on peripheral blood smear, along with a serum vitamin B12 level of 298 (normal range 203–339)?
Folate deficiency.

Question: A 21-year-old woman presents to your office looking pale, fatigued, and thin. She states that she has been trying to lose weight despite being underweight by today’s measurement. When discussing her oral intake you learn that she almost never eats any fresh fruits or vegetables. What is the likely diagnosis?
Folate deficiency.

Question: The best way to differentiate between vitamin B12 deficiency and folate deficiency is by which laboratory tests?
A normal vitamin B12 and a low serum folate level.

Question: What does treatment for folate deficiency consists?
Folic acid 1 mg/day orally with response seen within 5 to 7 days and resolution of all symptoms within 2 months.

Question: Why are red cell transfusions rarely required to treat iron deficiency anemia?
Nucleated red blood cells and reticulocytes appear in the blood stream within 72 hours after starting oral iron replacement.

What is the progression of biochemical and hematological events in iron deficiency anemia?
Decreased serum ferritin, then decreased serum iron and total iron binding capacity, followed by a fall in MCV and MCH, and a rise in RDW. Thrombocytosis may occur.

Question: A serum ferritin level less than 12 μ
g/L is notable for what diagnosis?
Iron deficiency anemia.

Question: What are common causes of iron deficiency anemia?
Dietary deficiency, decreased absorption, pregnancy, and blood loss.

Question: A 24-year-old woman who changed her dietary intake within the past 6 months to vegan complains of fatigue and tachycardia over the last 2 to 3 months. Recently, she has also developed palpitations and tachypnea on exertion. Her history is notable for heavy menstrual cycles. What laboratory test should you obtain?
Serum ferritin to evaluate for iron deficiency anemia.

Question: Early laboratory findings on blood smear looking for iron deficiency will include:
Anisocytosis.

Question: Later laboratory findings in iron deficiency include:
Normochromic, normocytic anemia.

Question: What is the most common human enzyme defect?
Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency.

Question: Clinical manifestations of individuals who inherit the common (polymorphic) forms of G-6-PD deficiency include:
No manifestations.

Question: Usually the anemia of G-6-PD is episodic and associated with stress most commonly from:
Drug administration, infection, and in some cases fava beans.

Question: What is the best therapy for a person with G-6-PD?
None, unless a hemolytic episode occurs. The use of transfusions is recommended in patients with brisk hemolysis along with good urine output. Avoiding drugs that might cause hemolysis should be considered but the drugs must not be avoided if the benefit outweighs the risk for the patient. In many cases patients with G-6-PD can tolerate many medications that previously were thought to be avoided.

Question: What syndrome is suggested in a child who is 6 months to 4 years old with an antecedent URI, fever, acute renal failure, microangiopathic hemolytic anemia (MAHA), and thrombocytopenia?
Hemolytic uremic syndrome.

What malignancy is most frequently associated with MAHA?
Gastric adenocarcinoma.

Question: What is the most common worldwide cause of hemolytic anemia?
Malaria.

Question: Hemolytic uremic syndrome (HUS) is associated with several etiologies that are grouped into two major classifications. It is important to recognize the differences because the clinical course and prognosis differ for each group. The two major groups are:

1. Diarrhea-associated HUS (D+HUS) 2. non–diarrhea-associated HUS (D-HUS).

Question: What is the most common cause of hemolytic uremic syndrome in the United States?
Diarrhea-associated HUS (D+HUS) from ingestion of E. coli 0157:H7.

Question: In a Coombs-positive hemolytic anemia patient, what should the differential include?
Autoimmune, drugs, infection, lymphoproliferative disease, and Rh or ABO incompatibility.

Question: What is the most common hemoglobin variant?
Hemoglobin S (valine substituted for glutamic acid in the sixth position on the β-chain).

Question: Which clinical crises are seen in patients with sickle cell disease?
- Vasoocclusive (thrombotic, painful) crisis
- Aplastic crisis
- Sequestration crisis
- Hemolytic crisis

Question: Which is the most common type of sickle cell crisis?
Vasoocclusive and is the hallmark of sickle cell crisis. It can occur almost daily to once per year.

Question: What percentage of patients with sickle cell disease have gallstones?
50% to 70% of adults have bilirubin gallstones and these may be found in children as young as 6 years.

Question: Current therapeutic recommendations for a patient in sickle cell crisis may include:
- Hydration
- Analgesia (if pain is associated)
- Oxygen (only beneficial if patient is hypoxic)
- Cardiac monitoring (if patient has history of cardiac disease or is having chest pain)

Question: What is the most commonly encountered sickle hemoglobin variant?
Sickle cell trait.

What is appropriate preventive immunization for a child with the sickle cell trait?
Pneumococcal conjugate and polysaccharide vaccine should be administered to all children who have sickle cell disease. Other routine immunizations, including yearly vaccination against influenza, should be provided.

Question: What is the difference between sickle cell anemia and sickle cell trait?
Sickle cell anemia is the homozygous form of the disease while sickle cell trait is the heterozygous form. People with the sickle trait are generally not anemic, are asymptomatic, and have a normal life span compared to those with the disease.

Question: How does a patient older than 12 months present with sickle cell disease?
By age 1 year, a severe hemolytic anemia may be present. The child will present with pallor, fatigue, and jaundice, and will be predisposed to developing gallstones. They may have splenomegaly due to sequestration and are at great risk for infection with encapsulated bacteria.

Question: A cure for β
-thalassemia major is possible with what treatment?
Allogeneic bone marrow transplant.

Question: Increased levels of hemoglobin A2 are found in what condition?
Thalassemia trait.

Question: RBC basophilic stippling occurs with what two disorders?
Thalassemia and lead poisoning.

Question: Hemosiderosis from chronic transfusion therapy for thalassemia can be successfully treated with:
Deferoxamine.

Question: Thalassemias are characterized by:
Microcytosis, family history, acanthocytes, and target cells.

Question: Thalassemias are classified into clinical categories based on outcomes. Identify the category and the outcome for each level:
1. Trait—without significant long-term clinical outcome
2. Intermedia—red blood cell transfusion requirements or other moderate clinical impact
3. Major—disorder is life-threatening

Question: In people with high transfusion requiring thalassemias, what is one of the primary laboratory values to monitor, along with primary therapy to apply?
Serum iron levels and iron chelation, using deferoxamine infusion.

COAGULATION DISORDERS

Question: What common drugs have been implicated to acquired bleeding disorders?
Ethanol, ASA, NSAIDs, warfarin, and antibiotics.

Question: Mucocutaneous bleeding, including petechiae, ecchymoses, epistaxis, GI, GU, and menorrhagia, indicate what coagulation abnormalities?
Qualitative or quantitative platelet disorders.

Question: Delayed bleeding and bleeding into joints or potential spaces, such as the retroperitoneum, suggest what type of bleeding disorder?
Coagulation factor deficiency (such as in hemophilia).

Question: What is primary hemostasis?
The platelet interaction with the vascular subendothelium that results in the formation of a platelet plug at the site of injury.

Question: What four components are required for primary hemostasis?
1. Normal vascular subendothelium (collagen)
2. Functional platelets
3. Normal von Willebrand factor (connects the platelet to the endothelium via glycoprotein Ib)
4. Normal fibrinogen (connects platelets to each other via glycoprotein IIB-IIIA)

Question: What is the end product of secondary hemostasis (coagulation cascade)?
Cross-linked fibrin.

Question: What is the principal physiologic activator of the fibrinolytic system?
Tissue plasminogen activator (tPA). Endothelial cells release tPA, which converts plasminogen, adsorbed in the fibrin clot, to plasmin. Plasmin degrades fibrinogen and fibrin monomer into fibrin degradation products (FDPs, once called fibrin split products) and cross-linked fibrin into D-dimers.

Question: How can an overdose of warfarin be treated? What are the advantages and disadvantages of each treatment?
Fresh frozen plasma (FFP) or vitamin K. However, if there are no signs of bleeding, temporary discontinuation may be all that is necessary. Treatment depends on the severity of symptoms, not the degree of prolongation of the prothrombin time (PT).
FFP:
advantages: rapid repletion of coagulation factors and control of hemorrhage.
disadvantages: volume overload, possible viral transmission.
Vitamin K-advantages: ease of administration.
disadvantages: possible anaphylaxis when given IV; delayed onset of 12 to 24 hours; effects may last up to 2 weeks, making anticoagulation of the patient difficult or impossible.

Which four hemostatic alterations are seen in patients with liver disease?
1. Decreased protein synthesis leading to coagulation factor deficiency
2. Thrombocytopenia
3. Increased fibrinolysis
4. Vitamin K deficiency

Question: What five treatments are available to bleeding patients with liver disease?

1. Transfusion with Packed RBCs (maintains hemodynamic stability)
2. Vitamin K
3. Fresh frozen plasma
4. Platelet transfusion
5. DDAVP (desmopressin)

Question: What hemostasis test is often prolonged in uremic patients?
Bleeding time.

Question: What treatment options are available to patients with renal failure and coagulopathy?
- Dialysis
- Optimize hematocrit (by recombinant human erythropoietin or transfusion with PRBCs)
- Desmopressin
- Conjugated estrogens
Cryoprecipitate and platelet transfusions if hemorrhage is life-threatening.

Question: What factors are deficient in classic hemophilia, Christmas disease (hemophilia b), and von Willebrand disease, respectively?
- Classic hemophilia: Factor VIII
- Christmas disease: Factor IX
- von Willebrand disease: Factor VIIIc and von Willebrand cofactor

Question: Vitamin K-dependent factors of the clotting cascade include:
X, IX, VII, and II. Remember 1972.

Question: What are the clinical complications of Disseminated intravascular coagulation (DIC)?
Bleeding, thrombosis, purpura fulminans, and multiorgan failure.

Question: Which three laboratory studies are most helpful in diagnosing DIC?
1. Prothrombin time (prolonged)
2. Platelet count (usually low)
3. Fibrinogen level (low)

There is a simultaneous activation of coagulation and fibrinolysis in what pathologic condition?
Disseminated intravascular coagulation.

Question: What are some common ischemic complications of DIC?
Renal failure, seizures, coma, pulmonary infarction, and hemorrhagic necrosis of the skin.

Question: What is the most important aspect of treating DIC?
Attempting to correct the underlying disorder (usually septic shock).

Question: A classic hemophiliac (hemophilia A) suffers a major head injury. What treatment should be given?
Give factor VIII.

Question: What pathway involves factors VIII and IX?
Intrinsic pathway.

Question: What effect does deficiency of factors VIII and IX have on PT and on PTT?
Deficiency leads to an increase in PTT.

Question: What agent can be used to treat mild hemophilia A and von Willebrand disease type 1?
1-Deamino-8-D-arginine vasopressin (DDAVP) induces a rapid rise in factor VIII levels.

Question: What are the most common sites for bleeding in patients with hemophilia?
Joints, muscles, and subcutaneous tissue.

Question: What is the only coagulation factor not synthesized by hepatocytes?
Factor VIII.

Question: What is the leading cause of death in hemophiliacs who received treatments prior to 1985?
AIDS.

Question: Hemophilia B is symptomatic in what patient populations?
Men; disease is X-linked.

Question: Patients with hemophilia B who are treated episodically early in childhood will develop what complications?
Severe arthritis and limited range of motion in affected joints.

Question: What is the mainstay of therapy for patients with factor XI disorders?
Fresh frozen plasma.

For patients with factor XI disorders that need a surgical procedure or are bleeding into mucosal surfaces, what therapy is regarded as mandatory for treatment?
Aminocaproic acid.

Question: In the thrombocytopenic patient, one unit of platelets will raise the platelet count by about how much?
One unit raises the platelet count by about 10,000/mm3.

Question: Below what platelet count is spontaneous hemorrhage likely to occur?
<10,000/mm3.

Question: What patients with thrombocytopenia are unlikely to respond to platelet infusions?
Those with antiplatelet antibodies (ITP or hypersplenism).

Question: What are the three major categories for the occurrence of thrombocytopenia?
Decreased production of platelets, increased destruction of platelets, and sequestration.

Question: Idiopathic thrombocytopenic purpura (ITP) disease course is different in children than in adults. How do the two differ?
In children, it is usually an acute disease often following an infection and is usually self-limited, while in adults it tends to take a more chronic disease course.

Question: Idiopathic thrombocytopenic purpura (ITP) is considered a secondary process when it is associated with an underlying autoimmune process. What are three common causes of ITP as a secondary process?
Systemic lupus erythematosus, and infections such as HIV and hepatitis C.

Question: Idiopathic thrombocytopenic purpura (ITP) is characterized by mucocutaneous bleeding and what on peripheral blood smear?
Often a very low platelet count and otherwise a normal peripheral blood smear.

Question: What is the characteristic bone marrow finding in idiopathic thrombocytopenic purpura (ITP)?
Increased or normal megakaryocytes.

Question: A patient is diagnosed with ITP. The patient has purpura noted on the extremities and a platelet count of 54 × 109/L, what is the most appropriate treatment for the individual?
Observation.

Question: What are the potential treatment modalities for ITP?
Gamma globulins (IVIG), steroids, splenectomy, anti-(RH) D, danazol, and antineoplastic drugs. However, the treatment modality should be tailored according to the patient’s condition as well as depending on bleeding issues; most patients require only observation of platelet counts, activity level of, and bleeding frequency.

Question: How do gamma globulin and steroids work in the treatment of ITP?
These block the uptake of antibody-coated platelets by splenic macrophages.

Under what circumstances is a bone marrow aspirate appropriate in the diagnosis and treatment of ITP?
If the patient is younger than 60 years, a bone marrow aspirate should be performed to rule out leukemia.

Question: What is the classic pentad of thrombotic thrombocytopenic purpura (TTP)?
1. Fever
2. Thrombocytopenia
3. Neurologic symptoms
4. Renal insufficiency
5. Microangiopathic hemolytic anemia (MAHA)

Question: What is the most common precipitating event for thrombotic thrombocytopenic purpura?
Pregnancy.

Question: What is the recommended treatment for acquired thrombotic thrombocytopenic purpura (TTP)?
Daily plasma exchange until several days after remission is obtained, denoted by normalization of platelet counts and LDH combined with clinical resolution of tissue ischemia and thrombosis.

Question: What are some signs and symptoms of thrombotic thrombocytopenic purpura (TTP)?
Thrombocytopenia, purpura, and microangiopathic hemolytic anemia. Patient with TTP presents with fever, fluctuating neurologic signs, and renal complications. If the disease goes untreated, it is almost uniformly fatal. Therapy includes steroids, splenectomy, plasmapheresis and exchange, and antiplatelet agents, such as dipyridamole and aspirin.

Question: What is the most common clinical presentation of TTP (thrombotic thrombocytopenic purpura)?
The most common clinical findings are thrombocytopenia and MAHA; however, most people present with neurologic symptoms including headache, confusion, cranial nerve palsies, coma, and seizures and through history purpura and other findings related to thrombocytopenia are noted.

Question: What is the most common inherited bleeding disorder?
von Willebrand disease.

Question: What is the currently approved mode of therapy for bleeding in patients with von Willebrand disease? What is the dose?
DDAVP, 0.3 μg/kg IV or subcutaneously every 12 hours for three to four doses.

Question: There are several types of von Willebrand disease; of these, which type is the most common?
von Willebrand disease type 1 accounts for 70% of clinically significant vWD.

Question: What is the most common clinical symptom with von Willebrand disease type 1?
Mucocutaneous bleeding. Other common findings are epistaxis, easy bruising, and hematoma.

What is von Willebrand disease?
An autosomal dominant disorder of platelet function. It causes bleeding from mucous membranes, menorrhagia, and increased bleeding from wounds. Patients with von Willebrand disease have less (or dysfunctional) von Willebrand factor.
von Willebrand factor is a plasma protein secreted by endothelial cells and serves two functions: (1) It is required for platelets to adhere to collagen at the site of vascular injury, which is the initial step in forming a hemostatic plug. (2) It forms complexes in plasma with factor VIII, which are required to maintain normal factor VIII levels.


MALIGNANCIES

Question: What are some signs and symptoms of leukemia?
Fever, fatigue and lethargy, petechia, bleeding, purpura, lymphadenopathy, hepatosplenomegaly, bone and joint pain, and pallor.

Question: What are some metabolic complications of leukemia?
Hypercalcemia, hyperuricemia, and syndrome of inappropriate antidiuretic hormone.

Question: How is hyperleukocytosis treated?
IV hydration, alkalinization, allopurinol, and antileukemic therapy.

Question: What percent of children with acute lymphoblastic leukemia (ALL) are cured by conventional chemotherapy?
70%.

Question: What is the most common malignancy for individuals younger than 15 years of age?
Acute lymphoblastic leukemia. It accounts for 23% of all cancers and 76% of all leukemia’s within this age group.

Question: What is the most common clinical presenting symptom for acute lymphoblastic leukemia?
Fever presents in approximately half of all patients with ALL. Other frequent findings are pallor, petechiae, and ecchymosis.

Question: The most common sites of extramedullary involvement for patients with ALL are:
Liver, spleen, and lymph nodes.

Question: Patients with ALL, considered at high risk for relapse following conventional chemotherapy, are considered for BMT. What patients are included in this group?
- Congenital or infants (< 1 year) with ALL
- Chromosomal translocation t (4,11), t (9,22)-Philadelphia, t (8,14)
- FAB L-3 morphology (Burkitt)
- WBC > 100,000
- Patients taking more than 1 month to achieve remission
- Patients who relapse while on chemotherapy
- Patients with more than one extramedullary site of relapse without a marrow relapse
- Patients with second and subsequent remissions

What do ALL and AML stand for?
Acute lymphoblastic leukemia and acute myelogenous leukemia.

Question: At what ages is ALL most common?
ALL peaks between ages 2 and 4 years. Incidence again rises in the sixth decade and reaches a second, smaller peak in the elderly.
Acute lymphoblastic leukemia (poor prognostic factors):

Lymphoblast high
Younger than 1 year

Male
Philadelphia marker
Hgb > 10-

Older than 10 years
Blood cells > 50,000

Lymph node enlarged
Anterior mediastinum masses
Spleen enlarged

T-cells
Induction > 4 weeks
Chromosome < 46

Question: AML accounts for what percentage of adult leukemias?
80%.

Question: What is the most common gene rearrangement in chronic myelogenous leukemia (CML)?
Translocation of abl 9 and bcr 22 commonly written t(9:22) or more commonly called the Philadelphia chromosome.

Question: What are common presenting symptoms in a patient with CML?
Easy fatigability, loss of sense of well-being, decreased tolerance to exertion, anorexia, abdominal discomfort, and early satiety (related to splenic enlargement), weight loss, and excessive sweating.

Question: The most common cytogenetic finding on blood smear of a patient diagnosed with CML is:
Philadelphia chromosome found in 90% of cases.

Question: The Ph+ chromosome rearrangement in CML is a positive long-term survival finding. In what disease is Ph+ chromosome rearrangement a poor prognostic finding with decreased median survival rate and early relapse?
Acute lymphocytic leukemia.

Question: What are early signs/findings for acute myelogenous leukemia?
Easy bruising, petechiae, epistaxis, gingival bleeding, conjunctival hemorrhages, and prolonged bleeding from skin injuries reflect thrombocytopenia.

Question: Most signs and symptoms of AML are related to what clinical feature?
Anemia.

What is the greatest risk factor in achieving a long-term remission in a patient diagnosed with AML?
Age, remission rates approach 90% in children, 70% in young adults, 50% in middle age, and 25% in old age.

Question: Non-Hodgkins lymphoma most often presents with what clinical finding?
Painless lymphadenopathy.

Question: A patient on chemotherapy for his Burkitt lymphoma is found to be hyperkalemic, hypocalcemic, hyperphosphatemic, and hyperuricemic. What is the presumptive diagnosis?
Tumor lysis syndrome.

Question: What is the malignant cell in Hodgkin disease?
The Reed-Sternberg cell.

Question: What are the peak age groups for Hodgkin disease?
15 to 34 years and over 60 years.

Question: What are the signs and symptoms of Hodgkin disease?
Painless supraclavicular or cervical lymphadenopathy, hepatomegaly, splenomegaly, unexplained fever, and night sweats.

Question: What are the indications for lymph node biopsy?
The indications for lymph node biopsy are imprecise. The decision to biopsy may be made early in a patient’s evaluation or delayed for up to 2 weeks. Prompt biopsy should occur if the patient’s history and physical findings suggest a malignancy; examples include a solitary, hard, nontender cervical node in an older patient who is a chronic user of tobacco, supraclavicular adenopathy, and solitary or generalized adenopathy that is firm, movable, and suggestive of lymphoma.

Question: How are childhood cases of non-Hodgkin lymphomas different from adult cases?
They grow rapidly, are rarely nodular, and are as likely to be T-cell lymphomas as B-cell lymphomas.

Question: What is the most common presentation of B-cell lymphomas?
Lymphadenopathy and hepatosplenomegaly.

Question: What is the most common presentation of T-cell lymphomas?
T cell lymphomas more likely present with cutaneous and bone marrow involvement.

Question: What condition should be suspected in a patient with multiple myeloma who presents with paraparesis, paraplegia, and urinary incontinence?
Acute spinal cord compression. This condition occurs primarily with multiple myeloma and lymphoma; it is also encountered with carcinomas of the lung, breast, and prostate.

Multiple myeloma is a malignancy of what cell line?
Plasma cells.

Question: What are the most common presenting complaints with someone who will be diagnosed with multiple myeloma?
Anemia, bone pain, and infection.

Question: What are common laboratory findings in a patient diagnosed with multiple myeloma?
Hypercalcemia, proteinuria, elevated sedimentation rate, or abnormalities on serum protein electrophoresis.

Question: What is the hallmark laboratory finding in multiple myeloma?
A paraprotein on serum protein electrophoresis (SPEP).

Question: What blood dyscrasias are currently being considered for treatment through BMT?
Fanconi anemia, thalassemia major, sickle cell disease, Diamond-Blackfan syndrome, and congenital sideroblastic anemia.

Question: What is graft versus host disease (GVHD)?
Engraftment of immunocompetent donor cells into an immunocompromised host, resulting in cell-mediated cytotoxic destruction of host cells if an immunologic incompatibility exists.

Question: When does acute GVHD present and what are the typical manifestations?
Acute GVHD typically becomes apparent between weeks 2 and 4 as the patient begins to engraft, and is characterized by erythroderma, cholestatic hepatitis, and enteritis.

Question: What is the clinical definition of chronic GVHD (cGVHD)?
Graft versus host disease lasting longer than 3 months posttransplant.

Question: How long is immunosuppressive treatment required for BMT recipients?
Usually 6 to 12 months or until a state of tolerance is attained.

Question: What are the most common types of infections seen posttransplant engraftment (day 0–30)?
Oral thrush, bacterial sepsis, catheter infections, fungal infections, pneumonia, and sinusitis.

Question: What are the most common types of infections seen in posttransplant postengraftment (day 30–100)?
CMV and EBV infection, viral hepatitis, toxoplasmosis, diffuse interstitial pneumonia, and cystitis.

Question: What are the most common types of infections seen posttransplant postengraftment (day 100–365)?
Varicella, herpes, CMV, toxoplasmosis, pneumocystis carinii pneumonia, viral hepatitis, and common bacterial infections.

What components of whole blood are used for transfusion?
- RBCs
- Platelets
- Plasma
- Cryoprecipitate

Question: How much will the infusion of 1 unit of PRBCs raise the hemoglobin and hematocrit in a 70-kg patient?
Hemoglobin: 1 g/dL. Hematocrit: 4%.

Question: What are the three conditions under which the transfusion of PRBCs should be considered?
1. Acute hemorrhage (blood loss > 1500 mL)
2. Surgical blood loss > 2L
3. Chronic anemia (Hgb < 7–8 g/dL, symptomatic, or with underlying cardiopulmonary disease)

Question: What five factors indicate the need to type and cross-match blood in the emergency department?
1. Evidence of shock from any cause
2. Known blood loss > 1000 mL
3. Gross GI bleeding
4. Hgb < 10; Hct < 30
5. Potential of surgery with further significant blood loss

Question: What are the main components of cryoprecipitate?
- Factor XIII
- von Willebrand factor
- Fibrinogen
- Cold-insoluble plasma proteins

Question: What is the first step in treating all immediate transfusion reactions?
Stop the transfusion.

Question: Since the advent of this testing method in blood products, viral infections are now considered negligible.
Nucleic acid amplification testing (NAT)

Question: What is the current recommended emergency replacement therapy for massive hemorrhage?
Type-specific, uncross-matched blood. Type O negative, although immediately lifesaving in certain situations, carries the risk of life-threatening transfusion reactions.

Question: In current practice, what blood components are routinely infused along with PRBCs in a patient receiving a massive transfusion?
None. The practice of routinely using platelet transfusion and fresh frozen plasma is costly, dangerous, and unwarranted.

What is the only crystalloid fluid compatible with PRBCs?
Normal saline.

Question: What is the incompatibility risk of typed blood, screened blood, and fully cross-matched blood?
The risk of incompatibility of ABO/Rh-compatible blood is 0.1% if the patient has never been transfused. The risk increases to 1.0% if the patient has had a previous transfusion. Adding a negative antibody screen decreases the risk to 0.06%. Fully cross-matched blood should carry a risk less than 0.05%.

Question: What is the most common blood group? What percentage of blood is Rh positive?
The most common blood group is type O; 45% of whites, 49% of African Americans, 79% of Native Americans, and 40% of Orientals are blood type O. Approximately 85% of the population is Rh positive and 15% Rh negative.

Question: What are the indications for the administration of FFP?
- Replacement of isolated factor deficiencies
- Reversal of coumadin effect
- Treatment of pathological hemorrhage in patients who have received massive transfusion
- Use in antithrombin III deficiency
- Treatment of immunodeficiencies

Question: What are the indications for cryoprecipitate administration?
Treatment of congenital or acquired fibrinogen and factor VIII deficiencies. Cryoprecipitate can also be administered prophylactically for nonbleeding perioperative or peripartum patients with congenital fibrinogen deficiencies or for von Willebrand disease that is unresponsive to desmopressin (DDAVP).

Question: Is it necessary to administer ABO-specific platelets?
The administration of ABO-specific platelets is not required because platelet concentrates contain few red blood cells. However, the administration of pooled platelet components of various ABO types can transfuse plasma-containing anti-A and/or anti-B, resulting in alloimmunization and a weakly positive direct antiglobulin test.

Question: What are the indications for platelet transfusion?
Platelets should be administered to correct thrombocytopenia or platelet dysfunction (thrombocytopathy). Perioperative factors to consider for the transfusion of platelets for counts between 50 and 100 × 109/L are the type of surgery, anticipated and actual blood loss, extent of microvascular bleeding, presence of medications (e.g., aspirin), and disorders (e.g., uremia) known to affect platelet function and coagulation. The prophylactic administration of platelets is not recommended in patients with chronic thrombocytopenia caused by increased platelet destruction (e.g., idiopathic thrombocytopenic purpura).

Question: What is the potassium load with transfusion?
It depends on the age of the blood. The potassium load steadily increases with time as extracellular potassium develops.

Question: What is the incidence of hemolytic transfusion reactions (HTR)?
1 in 38,000 units. The HTR is potentially life-threatening and often regarded the most serious complication of transfusions.

What are the types of HTRs and what is the pathophysiology of each one?
HTRs are divided in to two types of reactions: (1) intravascular hemolysis and (2) extravascular hemolysis or delayed hemolytic reaction.

Question: What is the treatment for HTRs?
The transfusion should be stopped immediately. Hypotension should be treated with fluids, inotropes, or other blood as appropriate. Renal output should be maintained with crystalloids, diuretics, or dopamine, as necessary. Component therapy should be used if DIC develops.

Question: What causes febrile reactions to blood and what are the incidences?
The febrile reaction is the most common mild transfusion reaction and occurs in 1% to 3% of transfusions. It is caused by alloantibodies (leukoagglutinins) to white blood cell, platelet, or other donor plasma antigens. Fever is presumably caused by pyrogens liberated from lysed cells. It occurs more commonly in previously transfused patients.

Question: What is transfusion-related acute lung injury (TRALI)?
TRALI is a form of noncardiogenic pulmonary edema, occurring within 2 to 4 hours after a transfusion. This reaction should be suspected in any patient who develops pulmonary edema after a transfusion in which volume overload is thought to be unlikely. Clinical signs of respiratory distress vary from mild dyspnea to severe hypoxia. It usually resolves within 48 hours in response to oxygen, mechanical ventilation, and other forms of supportive treatments.

Question: Petechia and bruising occur with platelet counts below what number? Internal hemorrhage occurs with count below what number?
<20,000/mm3 and <10,000/mm3, respectively.

Question: What electrolyte abnormality is commonly associated with the transfusion of packed RBCs?
Hypocalcemia secondary to citrate toxicity. Citrate, when rapidly infused, binds ionized calcium and therefore decreases the calcium level. Hyperkalemia may also develop, especially if the patient is in renal failure or if the blood products are old.

Question: Historically, what is the most common type of hepatitis transmitted through blood transfusions?
Hepatitis C.

Question: What is the universal type of blood donor?
Type Rh-negative blood with anti-A and anti-B titers of less than 1:200 in saline.

Question: What are the common presentations of a transfusion reaction?
Myalgia, dyspnea, fever associated with hypocalcemia, hemolysis, allergic reactions, hyperkalemia, citrate toxicity, hypothermia, coagulopathies, and altered hemoglobin function.

Question: What is the most serious transfusion reaction?
Hemolytic. Treat with aggressive fluid replacement and Lasix.

What is the most common transfusion reaction?
Fever.

Question: What is the most common cause of a coagulopathy in patients who require massive transfusions?
Thrombocytopenia.

Question: What blood product is given when the coagulation abnormality is unknown?
Fresh frozen plasma.

Question: In a nonacute noncardiac blood loss setting, what is the initial dose of blood given to children?
10 mL/kg of packed RBCs.

Question: What pathway does the PT measure? What factor is unique to this pathway?
Extrinsic pathway. Factor VII.

Question: What are the three major proteins that inhibit clotting?
Antithrombin III, protein C, and protein S.

Question: The deficiency of antithrombin III, protein C, or protein S increases the risk of what?
Venous thrombosis.

Question: Why should warfarin, as an initial treatment for venous thrombosis secondary to protein C deficiency, be avoided?
It may, by inhibiting the synthesis of protein C, lead to paradoxical hypercoagulability. Always treat with heparin before warfarin.

Question: What are the signs and symptoms of splenic sequestration crisis?
Pallor, weakness, lethargy, disorientation, shock, decreased level of consciousness, and enlarged spleen.

Question: What is the treatment of splenic sequestration crisis?
Rapid infusion of saline and transfusion of red cells or whole blood.

Question: What are the major complications of hereditary spherocytosis?
Hyperbilirubinemia in the newborn period, splenomegaly, and often gallstones.

Question: At what hematocrit level is the oxygen treatment capacity maximum?
It occurs at a hematocrit of 30%.

Question: What does prothrombin time (PT) measure? How is it performed?
PT measures the extrinsic and common pathways of the coagulation system. The time to clot formation is measured after the addition of thromboplastin. If the concentration of factors V, VII, IX, and X are significantly lower than usual, the PT may be prolonged.

What does activated partial thromboplastin time (PTT) measure? How is it performed?
PTT measures the intrinsic and common pathways of the coagulation cascade. After the blood sample is exposed to celite for activation and a reagent is added, the clot formation is measured. When factors II, V, VIII, IX, X, XI, XII, or fibrinogen are deficient, the PTT may be prolonged.

Question: What therapy should be initiated for a bleeding patient who is on warfarin and has a high PT?
D/C warfarin, followed by a water soluble form of vitamin K. Prescribe SQ and consider a test dose. If the bleeding is severe or in a dangerous location (i.e., the brain), fresh frozen plasma containing active factors X, IX, VII, and II should be given. (Remember: 1972.)


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