Heme (New) Flashcards

1
Q

A 53 yo has Auer rods and >20% blasts in bone marrow. WBC is >100K. Dx?

A

Acute Myeloid Leukemia (AML)

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2
Q

In what type of anemia would you find pancytopenia? What is that?

A
  • Aplastic Anemia

- All 3 blood cells low

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3
Q

What is the treatment for Primary Immune Thrombocytopenia in a patient with <30K platelets and is actively bleeding, has RFs, or does not respond to steroids (or was CI)?

A

IVIG

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4
Q

What labs are found in von Willebrand Disease (VWD)?

A
  • ↓ vWF
  • ↓ factor VIII (causes abnormal platelet function BUT normal platelet count)
  • prolonged BT
  • prolonged PTT (in 50%)
  • normal PT
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5
Q

What labs are found in Hemophilia A and B?

A
  • ↑ PTT
  • normal PT and platelets
  • ↓ Factor VIII or IX
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6
Q

What is the treatment for von Willebrand Disease (VWD)?

A

Desmopressin

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7
Q

What is the treatment for Hemophilia A and B?

A

Factor VIII or IX, cryoprecipitate

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8
Q

What is the appropriate treatment for a 52 yo man with severe aplastic anemia?

A

Anti-thymocyte globulin + Cyclosporine, and Prednisone

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9
Q

What is the treatment for Primary Immune Thrombocytopenia in a patient with <30K platelets and no bleed?

A

Steroids

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10
Q

How is anemia of chronic disease diagnosed?

A

Normal or ↑ Ferritin* + ↓TIBC*, ↓ Fe

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11
Q

A bone marrow biopsy shows hypocellular + fatty infiltration. What is the likely diagnosis?

A

Aplastic Anemia

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12
Q

Microcytic, hypochromic RBCs with ↓ Fe, ↑ total iron binding capacity (TIBC), ↓ ferritin levels would make you think:

A

Iron Deficiency Anemia

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13
Q

What are the lab findings for a B12 (Cobalamin) Deficiency?

A
  • ↑ homocysteine
  • ↑ methylmalonic acid (MMA)
  • hypersegmented neutrophils
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14
Q

With recurrent DVTs and pulmonary embolism, esp. young, one would think:

A

Factor V Leiden

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15
Q

What is the earliest finding in iron deficiency anemia?

A

↓ serum ferritin (low stores)

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16
Q

↑ MCV and megaloblasts. Dx?

A

Folate Deficiency

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17
Q

Suspect _____ if renal failure in kids with a prodrome of diarrhea associated with thrombocytopenia on the CBC

A

Hemolytic Uremic Syndrome (HUS)

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18
Q

In repeated spontaneous abortions, recurrent DVTs or CV events, one would think:

A

Antiphospholipid Antibody Syndrome

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19
Q

How is Factor V Leiden diagnosed and confirmed?

A

Activated Protein C assay. If (+) → confirm with DNA testing.

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20
Q

What is the only disorder that will cause an ↑ in mean corpuscular hemoglobin concentration (MCHC)?

A

Hereditary Spherocytosis

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21
Q

ART (Autoimmune hemolysis, Renal failure, Thrombocytopenia) + Fever + Neuro abnormalities = (FAT RN). Dx?

A

Thrombotic Thrombocytopenic Purpura (TTP)

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22
Q

↓ fibrinogen, ↑ PT/PTT/INR, severe thrombocytopenia; ↑ fibrinolysis (D-dimer). Dx?

A

Disseminated Intravascular Coagulation (DIC)

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23
Q

A 5yo male presents with purpura, arthritis, abdominal pain and hematuria. Mom reports a URI a couple weeks ago that has since cleared. What is the likely diagnosis?

A

Henoch-Schonlein Purpura

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24
Q

How is Henoch-Schonlein Purpura diagnosed?

A

Definitive: renal biopsy showing mesangial IgA deposits in granular pattern

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25
Q

How is Henoch-Schonlein Purpura treated?

A

ACE-I and early steroid therapy reduced proteinuria

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26
Q

Alpha Thalassemia is seen mostly in what ethnicities?

A

Asian > African > Mediterranean

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27
Q

Beta Thalassemia is seen mostly in what ethnicities?

A

Italian or Greek descent

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28
Q

How is Beta Thalassemia (minor) diagnosed?

A

Microcytic hypochromic cells, ↑ Hgb A2, target cells

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29
Q

How is Beta Thalassemia (MAJOR) diagnosed?

A
Severe anemia (Hgb ~6)
↓ MCV
↑ HgbF
Codocytes (target cells)
Hepatosplenomegaly
30
Q

X-ray showing hyperdense lines at metaphyses, also know as “___ lines” are due to:

A
  • “Lead”

- Lead Poisoning

31
Q

What is the treatment for lead poisoning?

A

Chelation therapy → PO Succimer or IV CaNa2EDTA (Calcium disodium edetate, given after Dimercaprol)

32
Q

How is Hereditary Spherocytosis diagnosed?

A

Osmotic fragility test

33
Q

The body stores enough folic acid to last:

A

4-5 months

34
Q

On a peripheral smear, what would be seen in Hereditary Spherocytosis?

A

Spherocytes and Howell-Jolly body

35
Q

How is Hereditary Spherocytosis treated? What about in newborns with jaundice?

A
  • Daily folic acid

- Phototherapy or exchange transfusion

36
Q

Labs: ↑ RBC mass, ↑ Hct, ↑ Hgb, abnormal proliferation of all 3 myeloid cell lines; Leukocytosis and thrombocytosis. Dx?

A

Polycythemia Vera

37
Q

Multiple, painless, peripheral lymphadenopathy; noncontiguous spread + GI Sxs in an immunocompromised pt is seen in:

A

Non-Hodgkin’s

38
Q

HIGH platelets, splenomegaly, pseudohyperkalemia, ↑ bleed time and Erythromelagia (burning and erythema of extremities) is seen in:

A

Thrombocythemia (Thrombocytosis)

39
Q

A 50 yo with splenomegaly and Philadelphia chromosome. Dx?

A

Chronic Myelogenous Leukemia (CML)

40
Q

↑ fasting Transferrin and Ferritin; ↓ TIBC. Dx?

A

Hemochromatosis

41
Q

Treatment for Hemochromatosis

A
  • Phlebotomy

- Deferoxamine chelation

42
Q

A 55yo male presents with RUQ pain, weakness, joint pains, sclera icteric and bronze extremities. What tumor marker should be checked in this patient?

A

Dx: Hemochromatosis

43
Q

(B symptoms) Fevers, night sweats, weight loss > 1 mo and painless, localized, single group of lymph nodes are found in:

A

Hodgkin’s

44
Q

Elderly pt. with bone + back pain refractory to treatment, labs showing monoclonal antibody spike and x-ray showing lytic lesions. Dx?

A

Multiple Myeloma

45
Q

What is the mutation for Polycythemia Vera?

A

Janus kinase 2 gene (JAK2)

46
Q

Child with lymphadenopathy, hepatosplenomegaly and >20% Blasts. Dx?

A

Acute Lymphocytic Leukemia (ALL)

47
Q

What d/o has increased systemic absorption of iron, resulting in iron saturation and fibrosis in various organs?

A

Hemochromatosis

48
Q

What is the treatment for Thrombocythemia (Thrombocytosis)?

A
  • Antiplatelet (Anagredlide and low-dose ASA)

- Hydroxyurea (severe)

49
Q

What is the treatment for Chronic Myelogenous Leukemia (CML)?

A
  • Curative: Allogeneic hematopoietic stem cell transplantation (HSCT)
  • Imatinib
50
Q

A 50 yo with isolated lymphocytosis (>20 K) and smudge cells. Dx?

A

Chronic Lymphocytic Leukemia (CLL)

51
Q

A 67 yo presents with SOB, fatigue and easy bruising. Labs show ↓ RBCs, WBCs and platelets, ↑ MCV, ↑ red cell width (RCW), ↓ retic count and ↑ blasts. What is the likely diagnosis?

A

Myelodysplastic Syndrome

52
Q

What diagnosis present with splenomegaly?

A
Autoimmune Hemolytic Anemia
Polycythemia Vera
Thrombocythemia (Thrombocytosis) 
Acute Myeloid Leukemia (AML)
Chronic Myelogenous Leukemia (CML)
53
Q

What diagnosis present with hepatosplenomegaly?

A
Anemia
Acute Lymphocytic Leukemia (ALL)
Beta Thalassemia Major (Cooley’s anemia)
Cirrhosis 
Toxoplasmosis 
Cytomegalovirus 
Histoplasmosis (immunodeficient)
54
Q

What is prophy for sickle cell disease in kids and when is it started?

A
  • Penicillin V (start @ 2 mo until 5yo)

- Folic acid (start @ 1 yo)

55
Q

A patient has a platelet count of 10,000, prolonged prothrombin level, and the presence of fibrin split products. Dx?

A

Disseminated Intravascular Coagulation (DIC)

56
Q

Which diagnoses have a (+) Coombs test?

A
  • Autoimmune Hemolytic Anemia

- Primary Immune Thrombocytopenia (formerly ITP)

57
Q

What 2 conditions present with fragmented RBCs on peripheral smear?

A

Disseminated intravascular coagulation (DIC) and Thrombotic thrombocytopenic purpura (TTP)

58
Q

What d/o is caused by defective synthesis of protoporphyrins and causes iron to accumulate in mitochondria?

A

Sideroblastic Anemia

59
Q

What is the treatment for Pernicious Anemia?

A

IM Cobalamin (B12) Q month

60
Q

With an elevated prothrombin time (PT) but a normal partial prothrombin time (PTT), what is the most appropriate pharmacotherapy?

A

Vitamin K

61
Q

What is a know RF for Myelodysplastic Syndrome?

A

Previous radiation

62
Q

What are Prussian blue stains use for?

A

Evaluate iron stores and check for Sideroblastic Anemias

63
Q

In what condition is Rouleaux formation is seen?

A

Multiple Myeloma (Plasmacytoma)

64
Q

In Sickle Cell Disease, what labs and smear are seen to diagnose?

A

↑ reticulocytes
↓ Hct, Hgb
Smear: Howell-Jolly body

65
Q

What disorders need cryoprecipitate if they are bleeding?

A
  • von Willebrand disease
  • Hemophilia A
  • Disseminated intravascular coagulation (DIC)
66
Q

What disorder is shown to have isolated lymphocytosis in asymptomatic patient?

A

Chronic Lymphocytic Leukemia (CLL)

67
Q

What are some causes of a prolonged Partial Prothrombin Time (PTT)?

A
  • vWD
  • Hemophilias
  • Disseminated Intravascular Coagulation (DIC)
  • Newborn Hemorrhagic Disease / Vitamin K Deficiency
  • Heparin use
68
Q

What are some causes of a prolonged Prothrombin Time (PT)?

A
  • Disseminated Intravascular Coagulation (DIC)
  • Newborn Hemorrhagic Disease / Vitamin K Deficiency
  • Warfarin use
69
Q

What electrolyte abnormality is most commonly seen in a patient with multiple myeloma?

A

Hypercalcemia

70
Q

What is the treatment for Tumor Lysis Syndrome?

A

Allopurinol and IVF