Hematology and Oncology USMLE

Pregunta	Respuesta
From which embryonic layer are angioblasts derived?	Mesoderm.
Name the site of red blood cell production during each of the following developmental phases.	First trimester-yolk sac Second trimester-liver and spleen Third trimester- central and peripheral skeleton Postpartum-Axial skeleton
What is the most common inherited hypercoagulable state?	Factor V leiden.
The factor V leiden mutation inhibits factor V cleavage by which protein?	Protein C
What is the effect of estrogen, endogenous or exogenous, on thrombosis?	Estrogen induces a hypercoagulable state.
What clotting factors require vitamin K for their synthesis?	Factors 2, 7, 9, 10 and protein C and S.
What common laboratory test is used to access the intrinsic coagulation system?	Partial thromboplastin time (PTT)
What common laboratory test is used to assess the extrinsic coagulation pathway?	Prothrombin time (PT)
What commonly used anticoagulant interferes with the extrinsic pathway?	Warfarin.
The use of the chelator, ethylene diamine tetra acetate (EDTA), as an anticoagulant, in tubes is designed to inactivate what factor in the blood?	Calcium
What inhibitor of the coagulation cascade inactivates factor Va and VIIIa?	Protein C
What are the two major functions of von Willebrand factor?	Transport of factor VIII Linkage of platelets and collagen.
Dysfunction of what clotting component results in mucous membrane hemorrhage, petechiae, purpura, and prolonged bleeding time?	Platelet abnormalities
Dysfunction of what clotting component results in hemarthroses, purpura, and prolonged PT and/or PTT?	Coagulation factor abnormalities
Name the coagulopathy associated with the following clinical and pathologic features: Most common hereditary bleeding disorder	von Willebrand disease
Most common type of hemophilia	Hemophilia A (factor VIII deficiency)
Most common cause of acquired platelet dysfunction	Aspirin use
Autosomal dominant (AD) disorder causing increased bleeding time	von Willebrand disease
Autosomal recessive defect in platelet adhesion caused by GpIIb/IX	Bernard-Soulier disease
AR defect in platelet aggregation caused by deficiency of GpIIb/GpIIIa, a membrane receptor responsible for binding fibrinogen.	Glanzmann thrombasthenia
Common presentation includes hemarthroses and easy bruising, increased PTT, normal PT	Hemophilia A and B.
Prolonged bleeding time with normal platelet count, normal PT and increased PTT	von Willebrand disease
Prolonged PT, PTT, increased bleeding time, thrombocytopenia, presence of fibrin split products	Disseminated intravascular coagulation (DIC)
Normal PT, PTT, normal platelet count, increased bleeding time	Aspirin use
Syndrome characterized by antiplatelet antibodies (commonly anti-Ib-IX or IIb-IIIa), often post-viral infections	Idiopathic thrombocytopenia (ITP)
Syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever and neurologic symptoms	Thrombotic thrombocytopenic purpura (TTP)
Widespread hyaline microthrombi in arterioles and capillaries causing schistocytes and helmet cells.	Microangiopathic hemolytic anemia
Splenomegaly, epistaxis, petechiae, generalized tender lymphadenopathy, serum antibodies (+)	SLE-autoimmune thrombocytopenia
List five of the most common causes of microcytic anemia	Iron deficiency, lead poisoning, chronic disease, sideroblastic, thalassemia.
List four of the most common causes of normocytic anemia	Sickle cell anemia, Aplastic anemia, acute blood loss, hemolytic anemia.
List five of the most common causes of macrocytic anemia	Liver disease Vitamin B12 deficiency Folate deficiency Alcoholism Hypothyrodism
What is the primary site of iron absorption? Vitamin B12? Folate?	Iron absorption- Duodenum Vitamin B12-Terminal ileum (need intrinsic factor) Jejunum-Folate
What is the major cause of iron deficiency anemia in adults?	Menorrhagia or gastrointestinal (GI) bleeding (colon cancer, ulcers)
What are two common clinical complaints in an anemic patient?	Dyspnea on exertion Fatigue
Which amino acid substitution in the B globin gene is most commonly seen in sickle cell anemia?	Glu6-Val
What is the major type of hemoglobin in homozygous sickle cell anemia?	HbS
In sickle cell disease, what is the mechanism of ischemic necrosis of the bones, lungs, liver, brain, spleen or penis?	Decreased oxygen tension, abnormal RBCs sickle, microvascular occlusions
What is the major regulatory enzyme in heme biosynthesis?	Aminolevulinate (ALA) synthase
What two common enzyme deficiencies can cause hemolytic anemia?	Glucose 6 phosphate dehydrogenase (G6PD) Pyruvate kinase.
What hemolytic anemia is associated with exposure to oxidant stress?	G6PD deficiency
What two common RBC membrane defects can cause hemolytic anemia?	1) Hereditary spherocytosis 2) Paroxysmal noctural hematuria (PNH)
What common cause of atypical pneumonia is associated with cold immune hemolytic anemia?	Mycoplasma pneumoniae
What type of anemia is caused by lack of intrinsic factor (IF) secretion by gastric parietal cells?	Pernicious anemia (vitamin B12 deficiency)
What two autoimmune diseases of the GI tract can cause megaloblastic anemia?	Pernicious anemia (Due to lack of intrinsic factor production) Crohn disease of the distal ileum (due to lack of the intrinsic factor-B12 complex reabsorption)
What parasites are capable of causing megaloblastic anemia?	Diphyllobothrium latum (by depleting B12) and Giardia lambia (by depleting folate)
How does gastric resection cause megaloblastic anemia?	Parietal cells, which are responsible for intrinsic factor production may be removed when the gastric fundus is resected.
What type of malignancy is associated with pernicious anemia?	Gastric carcinoma
Name three medications capable of causing autoimmune hemolytic anemia?	Penicillin, Cephalosporins, Quinidine
What types of malignancies are associated with autoimmune hemolytic anemia?	Leukemias and lymphomas.
What laboratory tests are seen in hemolytic anemia?	Increased in unconjugated bilirubin, increased urine urobilinogen, decreased hemoglobin, hemoglobinuria, decreased haptoglobin, hemosiderosis.
Name two commonly used medications that can cause aplastic anemia	Nonsteroidal anti-inflammatory drugs (NSAIDs), Chloramphenicol
What test is used to monitor anticoagulation in a patient treated with heparin?	PTT
What test is sued to monitor anticoagulation in a patient treated with warfarin?	PT
What happens to the total iron binding capacity, serum iron concentration and percent saturation of transferrin in each of the following diseases?	Iron deficiency anemia: Increased TIBC, Decreased serum iron, Decreased % saturation Anemia of chronic disease:-Decreased TIBC, Decreased serum iron, normal saturation Iron overload-Normal TIBC, Increased serum iron, maximal saturation
Name the types of anemia associated with the following clinical and pathologic features:	Most common type of anemia-Iron deficiency anemia Abnormal Schilling test-Pernicious anemia
ABO incompatility, lymphoid neoplasm, Raynaud phenomena, anti-i antibodies	Cold autoimmune hemolytic anemia
Atrophic glossitis	Pernicious anemia
Autosplenectomy	Sickle cell anemia
Basophillic stipling of erythrocytes, blue/gray discoloration at gumline, wrist/foot drop	Anemia from lead poisoning
Celiac sprue	Folate deficiency anemia (megaloblastic)
Chronic atopic gastritis	Pernicious anemia
Colon cancer	Iron deficiency anemia (early) and anemia of chronic disease (late)
Crescent-shaped erythrocytes and Howell-Jolly bodies	Sickle cell anemia
Deficiency of a or B globin gene synthesis	Thalassemia
Deficiency of decay accelerating factor	Paroxysmal noctural hemoglobinuria.
Demyelination of the dorsal and lateral tracts of the spinal cord	Pernicious anemia
End stage liver disease	Macrocytic anemia
Helmet cells, burr cells, triangular cells	Microangiopthic anemia (secondary to DIC, thrombocytopenic purpura, hemolytic-uremic syndrome (TTP-HUS), or mechanical heart valves)
Hypersegmented polymorphonuclears (PMNs)	Vitamin B12 or folate deficiency anemia
Increased serum lactate dehydrogenase	Hemolytic anemia
Microcytosis, atopic glossitis, esophageal webs (Plummer-Vinson syndrome)	Iron deficiency anemia
Pancytopenia and fatty infiltration of bone marrow	Aplastic anemia
Systemic lupus erythematosus (SLE), chronic lymphocytic leukemia (CLL), lymphomas, drugs +direct Coombs test (due to IgG autoantibodies)	Warm autoimmune hemolytic anemia
AD deficiency of spectrin, positive osmotic fragility test	Heriditary spherocytosis
What are the two major categories of lymphoma?	Hodgkin disease Non-hodgkin lymphoma
Interleukin (IL-5) secreting Reed Sternberg cells	Hodgkin disease
Commonly arises from B cells	Non-hodgkin lymphoma
Constitutional symptoms including both fever, night sweats weight loss	Both
Mediastinal lymphadenopathy, contiguous spread	Hogkin disease
Many cases associated with Epstein Barr virus	Hodgkin disease
Peripheral lymphadenopathy, noncontiguous spread	Non-hogkin lymphoma
Bimodal age distribution, but most common in young men	Hodgkin disease
Peak incidence from 20 to 40 years of age	Non hodgkin lymphoma
Associated with immunosuppression including AIDS	Non hodgkin disease
Painful lymphadenopathy with alcohol consumption	Hodgkin disease
Most common hodgkin disease	Nodular sclerosis
Abundance of RS cells	Mixed cellularity
Lacunar cells and collagen binding	Nodular sclerosis
Commonly seen in older patients with Hodgkin disease	Mixed cellularity
Widely disseminated disease with poor prognosis	Lymphocyte depletion
Most common in females	Nodular sclerosis
Abundance of lymphocytes	Lymphocyte predominance
Commonly seen in men 35 years presenting with cervical or axillary lymphadenopathy	Lymphocyte predominace
Higher proportion of RS cells relative to lymphocytes	Lymphocyte depletion
Overexpression of cyclin D1	Mantle cell lymphoma
Starry-sky appearance on histopathology	Burkitt lymphoma
Clinically similar to CLL: characterized by nodules of small lymphocytes	Small cell lymphocytic lymphoma
Older adults with BCL2 gene mutation	Follicular lymphoma
Often appear at extranodal sites and can cause small bowel obstruction	Diffuse large cell lymphoma
Child presenting with enlarging mandibular mass	Burkitt lymphoma
Common in children who present with a mediastinal mass and a syndrome similar to that of acute lymphocytic leukemia (ALL)	Lymphoblastic lymphoma
TdT+ lymphocytes	Precursor B or T cell acute lymphoblastic leukemia/lymphoma
Endemic in Africa and associated with Epstein Barr virus	Burkitt lymphoma
0-14 years old	acute lymphocytic leukemia
15-39 years old	Acute myelogenous leukemia
40 to 59 years old	Acute myelogenous leukemia and chronic myelogenous leukemia
More than 60 years old	Chronic lymphoblastic leukemia
Very high white cell counts, often more than 200,000	Chronic myelogenous leukemia
Isolated lymphocytosis	chronic lymphoblastic leukemia
TdT+ lymphoblasts	Acute lymphocytic leukemia
Name the four chronic myeloproliferative disorders	Chronic myelogenous leukemia Polycythemia vera Essential thrombocytosis Myelofibrosis with myeloid metaplasia

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