Hemepathology Flashcards

1
Q

How does erythropoietin promote the survival of early stem cell progenitors?

A

By inhibiting apoptosis

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2
Q

What is a major contributor causing decreased folate intake?

A

Poor diets and commonly alcoholism

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3
Q

What causes pernicious anemia?

A

AutoAbs to gastric parietal cells and intrinsic factor

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4
Q

What conditions do autoAbs of gastric parietal cells lead to?

A

Atrophic gastritis and achlorhydria

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5
Q

What does the peripheral blood smear show in megaloblastic anemia?

A

Macrocytosis and hypersegmented neutrophils

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6
Q

What is megaloblastic maturation characterized by?

A

Cellular enlargement and asynchronous maturation of nucleus and cytoplasm in bone marrow precursors of all lineages

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7
Q

What is aplastic anemia?

A

Disorder of pluripotent SCs that leads to BM failure

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8
Q

In aplastic anemia, what is the general shift of cellular components in the body?

A

Thrombocytopenia, anemia, leukopenia (primary granulocytopenia) with a shift towards lymphocytes and plasma cells. Erythroid, myeloid, and megakaryocytes lineages decrease.

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9
Q

Describe the RBCs in iron deficiency anemia

A

Hypochromic, microcytic

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10
Q

Thin woman with menorrhagia - what is the likely anemia Dx?

A

Iron deficiency microcytic anemia

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11
Q

A woman comes in with microcytic anemia and a chronic upper abdominal pain. Why can it be concluded that she is having improper heme synthesis leading to her anemia?

A

She is not getting adequate iron from her diet/she is bleeding into her stomach and using up her iron stores. Bottom line, her iron stores are low and she can’t make good heme.

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12
Q

What is the main culprit in hereditary spherocytosis?

A

Genetic inheritance that results in a poor RBC cytoskeleton (lack of proteins: spectrin, protein 4.2, band 3

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13
Q

What test is abnormal in hereditary spherocytosis?

A

Osmotic fragility test

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14
Q

What is a common complication of those with hereditary spherocytosis? Mechanism?

A

Cholelithiasis; Extravascular hemolysis of the sphereocytes occurs causing hyperbilirubinemia. The increased bilirubin causes formation of gallstones

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15
Q

Describe appearance of gallstones composed of bilirubin

A

Pigmented

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16
Q

What is hereditary elliptocytosis? What proteins are involved in this disorder?

A

Heterogenous group of inherited disorders involving the erythrocyte cytoskeleton featuring a horizontal abnormality with the cytoskeleton. Spectrin (self assembly), spectrin-ankyrin binding, protein 4.1, and glycophorin C.

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17
Q

What would be a tip regarding the hemoglobin that a nearly year-old child had beta-Thalassemia?

A

The child only has feta hemoglobin; His blood smear shows hypochromic/microcytic RBCs with anisocytosis (unequal size RBCs), poiklocytosis (abnormal shaped RBCs) and target cells

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18
Q

Describe the mechanism of splenomegaly in a patient with Beta-Thalassemia

A

The patient is anemic and therefore there is bone marrow hyperplasia in an attempt to pump out more RBCs. Extramedullary erythropoiesis spreads to the spleen resulting in splenomegaly.

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19
Q

If a patient presented with anemia and when tested had a mild increase in hemoglobin A2, what condition would you suspect?

A

Heterozygous Beta-Thalassemia

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20
Q

A mother who has a history of several miscarriages delivers a 36 week neonate who is pale and anemic. What is the likely cause of the anemia and what do you expect to see on the blood smear?

A

The mother likely has an IgG to an antigen of the fetus and causes compliment-mediated lysis of the neonates RBCs; On blood smear, you would expect to see immature erythroid precursors containing nuclei.

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21
Q

What lab results would you expect in a patient with polycythemia vera?

A

High Hb, High Hct, Hypercellular bone marrow, Depletion of marrow iron stores

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22
Q

What condition is a person with Polycythemia vera at risk for?

A

Cerebrovascular accident because of the increased viscosity of the blood

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23
Q

Mnemonic to remember what kind of sickle cell condition a person with HbS has:

A

The “S” stands for sickle - sickle cell disease

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24
Q

What is the cause of broad systemic pain in a person with sickle cell disease?

A

Abundant ischemia of the uvasculature results in pain in chest, abdomen and bones

25
Q

What is a common complication following an autosplenectomy?

A

Susceptibility to infections by encapsulated bacteria (especially S. pneumoniae).

26
Q

What virus causes aplastic crisis in patients with sickle cell disease?

A

Parvovirus B19

27
Q

Describe pathogenesis of chronic disease leading to anemia

A

The body sequesters iron in the bone marrow to keep it from bacteria

28
Q

What is acanthocytosis and what condition causes it?

A

Defect within the lipid bilayer of an RBC resulting in spiny projections from the surface. It is caused by chronic liver failure

29
Q

What are typical cellular presentations of myelodysplastic syndrome?

A

Anemia, throbocytopenia, and neutropenia. Furthermore, ringed sideroblasts are also observed.

30
Q

What is the Prussian blue stain used to observe?

A

Iron-laden sideroblasts

31
Q

What is the cause for myelodysplastic syndrome?

A

Clonal stem cell defect

32
Q

What is Fanconi Anemia?

A

Genetic defect that results in an inability to repair DNA and often results in acute myeloid leukemia

33
Q

What is the Schilling test?

A

Test for VitB12 absorption

34
Q

What disease are Heinz bodies indicative of?

A

G6PD Deficiency

35
Q

What is the cause for myelodysplastic syndrome?

A

Clonal stem cell defect

36
Q

What is Fanconi Anemia?

A

Genetic defect that results in an inability to repair DNA and often results in acute myeloid leukemia

37
Q

What is the Schilling test?

A

Test for VitB12 absorption

38
Q

What disease are Heinz bodies indicative of?

A

G6PD Deficiency

39
Q

It what cancer are Auer Rods seen?

A

Acute promyelocytic leukemia

40
Q

What do promyelocytes eventually become?

A

Neutrophils

41
Q

What is the translocation that occurs in APL?

A

t(15;17); PML(15) and RAR (17) fuse

42
Q

In what malignancy are smudge cells seen on peripheral blood smear?

A

Chronic lymphoblastic leukemia

43
Q

In what population do 75% of B-ALL cases occur?

A

Children < 6 yo

44
Q

In what disease are owl-eye cells seen?

A

Hodgkin lymphoma

45
Q

Which type of Hodgkin Lymphoma is most common?

A

Nodular sclerosis

46
Q

What is the most common and most important complication of multiple myeloma leading to death?

A

Amyloid nephropathy

47
Q

A patient is observed to have marrow fibrosis and prominent megakaryopoiesis and granulopoiesis. What is the likely Dx?

A

Chronic idiopathic myelofibrosis

48
Q

Genetic finding in person with follicular lymphoma?

A

Bcl-2 overexpression due to 14:18 transolcation

49
Q

What type of lymphoma is Burkitt’s characterized as in Rubins?

A

Large B-Cell Lymphoma

50
Q

What leukemia cells exhibit Tartrate Resistant Acid Phosphatase marker?

A

Hairy Cell Leukemia cells

51
Q

What conditions are marginal zone lymphomas associated with?

A

H. pylori, Hashimoto, Sjoegrens

52
Q

In what neoplastic disease are Dutcher and Russel bodies found? What are they?

A

Waldenstrom’s; Igs!

53
Q

9 yo girl with petechiae and a platelet count of 20K had flu-like illness a week earlier and her BM showed megakaryocytosis. Platelets normal after 2 months. Dx?

A

Idiopathic Thrombocytopenic Purpura

54
Q

What is the pentad of thrombotic thrombocytopenic purpura?

A

Thrombocytopenia, Microangiopathic Hemolytic Anemia, Fever, Renal Impairment, Neuro Syx

55
Q

What two tests are utilized to Dx Paroxysmal Nocturnal Hemoglobinuria?

A

Ham acid test and sucrose test

56
Q

What condition is characterized by uncontrolled proliferation of megakaryocytes?

A

Essential thrombocythemia

57
Q

What is the normal count for platelets?

A

150-450K

58
Q

What disease is characteristic of having myeloblasts in the peripheral blood?

A

Acute Myeloid Leukemia

59
Q

What is suppurative lymphadenitis?

A

Lymph node swelling a site draining an infection