Heme Flashcards

1
Q

preferred anticoagulant for immediate anticoagulation

A

heparin

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2
Q

long-term anticoagulation

A

warfarin

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3
Q

what allows RBC to change shape in vessels

A

spectrin

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4
Q

lead poisoning

A

basophilic stippling

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5
Q

G6PD deficiency

A

Heinz bodies and bite cells

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6
Q

DIC

A

schistocytes

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7
Q

abetalipoproteinemia

A

acanthocytes (spur cells)

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8
Q

asplenia

A

howell-jolly bodies and target cells

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9
Q

liver disease

A

acanthocytes and target cells

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10
Q

bone marrow infiltration (myelofibrosis)

A

teardrop cell

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11
Q

Target cells

A

THAL

Thallasemia, HbC disease, Asplenia, Liver disease

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12
Q

absence of HGPRTase

A

Lesch-Nyhan

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13
Q

deficiency of aldolase B

A

fructose intolerance

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14
Q

deficiency of cystathionine synthase

A

homocystineuria

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15
Q

galactose-1-phophate uridyl transferase deficiency -> intellectual disability, HSM, cataracts

A

galactosemia

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16
Q

deficiency of tyrosinase

A

albinism

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17
Q

location of fetal erythropoiesis

A
Young livers synthesize blood
yolk sac
liver
spleen
bone marrow
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18
Q

different causes of polycythemia

A

increased EPO (tumors)
chronic hypoxia
polycythemia vera
trisomy 21

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19
Q

lead poisoning

A

hematologic: microcytic anemia, basophilic stippling, ringed sideroblasts
non-hematologic: encephalopathy, memory loss, HA, foot/wrist drop, lead lines, abdominal colick, renal failure

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20
Q

lead poisoning affects which enzymes

A

ferrochelatase and ALA dehydratase

leads to accumulation of protoporphyrin and d-ALA

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21
Q

acute intermittent porphyria

A

inhibits porphobilinogen deaminase (uroporphyrinogen-1-synthase)
accumulates porphobilinogen, d-ALA, and uroporphyrin
5 P’s:
painful abdomen, port-wine urine, polyneuropathy, psychological disturbances, precipitated by drugs

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22
Q

treatment of acute intermittent porphyria

A

glucose and heme (inhibits ALA synthase)

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23
Q

porphyria cutanea tarda

A

uroporphyrinogen decarboxylase
accumulates uroporphyrin
blistering cutaneous photosensitivity, hypertrichosis, facial hyperpigmentation, Hep C and alcoholism

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24
Q

RLS of Heme synthesis

A

d-ALA synthase (d-aminolevulinic acid)

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25
Q

diagnose beta-thalassemia minor

A

HbA2 on electrophoresis

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26
Q

lab findings of iron def:

A

decreased iron and ferritin, increased TIBC

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27
Q

what should be ruled out in 50 yo pt with new-onset iron deficiency anemia

A

colon cancer

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28
Q

microcytic anemia + swallowing difficulty + glossitis

A

Plummer-Vinson syndrome

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29
Q

microcytic anemia + >3.5% HbA2

A

B-thalassemia minor

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30
Q

megaloblastic anemia not correctable by B12 or folate

A

orotic aciduria

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31
Q

megaloblastic anemia along with peripheral neuropathy

A

B12 deficiency

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32
Q

microcytic anemia + basophilic stippling

A

lead poisoning

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33
Q

microcytic anemia reversible with B6

A

sideroblastic anemia

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34
Q

HIV-positive patient with macrocytic anemia

A

Zidovudine induced

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35
Q

Normocytic anemia and elevated creatinine

A

chronic renal disease

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36
Q

causes of hypochromic, microcytic anemias

A

iron deficiency, thalassemia, lead poisoning

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37
Q

hypersegmented neutrophils

A

megaloblastic anemia (B12 or folate)

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38
Q

skull x-ray shows a “hair-on-end” appearance

A

marrow hyperplasia

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39
Q

basophilic stippling of RBC

A

lead poisoning

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40
Q

findings associated with hereditary spherocytosis

A

anemia, jaundice, pigmented gallstones, splenomegaly, spherocytes
+ osmotic fragility test
(-) Coombs test

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41
Q

Ham’s test

A

Paroxysmal nocturnal hemoglobinuria

cells lyse when placed in acid (now use flow cytometry to look for no CD55/CD59)

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42
Q

Heinz bodies

A

G6PD deficiency

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43
Q

painful cyanosis of fingers and toes, with hemolytic anemia

A

cold autoimmune hemolytic anemia

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44
Q

red urine in the morning, and fragile RBC

A

paroxysmal nocturnal hemoglobinuria

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45
Q

basophilic nuclear remains in RBC

A

howell-jolly body

46
Q

autosplenectomy

A

sickle cell disease

47
Q

drug used to treat sickle cell disease

A

hydroxyurea

48
Q

what does ADP and TxA2 do

A

ADP: induces GpIIb/IIIa expression on platelets
TxA2: promotes crosslinking of GpIIb/IIIa by fribrinogen

49
Q

cause of ITP

A

Ab against GpIIb/IIIa

lab shows increased megakaryocytes

50
Q

Bernard-Soulier disease

A

defective GpIb

51
Q

mom bleeds profusely from vagina and later gums after delivery

A

DIC

52
Q

DIC

A

increased BT, PT, PTT, D-dimers
decreased platelets and fibrinogen
STOP Making Thrombi
Sepsis, trauma, obstetrics, pancreatitis, malignancy, transfusions

53
Q

antiplatelet antibodies

A

immune thrombocytopenia (ITP)

54
Q

most common inherited bleeding disorder

A

von Willebrand disease

55
Q

HUS-TTP

A

HUS triad: hemolysis (microangiopathic), renal insufficiency, thrombocytopenia
TTP adds neurologic symptoms and fever

56
Q

TTP

A

defect in ADAMTS 13 that causes failure to breakdown vWF multimers

57
Q

Glanzmann’s thrombasthenia

A

deficient GpIIb/IIIa

lab shows no platelet clumping

58
Q

age distributions for Hodgkin and non-hodgkin lymphoma

A

Hodgkin peak at 20 and >65

non-Hodgkin variable but 20-40 common

59
Q

most common non-Hodgkin lymphoma in adults and children

A

adult: diffuse large B cell lymphoma
children: lymphoblastic lymphoma

60
Q

most common lymphoma in US

A

diffuse large B-cell

61
Q

Reed-Sternberg cells

A

Hodgkin lymphoma

62
Q

particularly associated with EBV

A

Burkitt, also Hodgkin lymphoma

63
Q

associated with long-term celiac disease

A

intestinal

64
Q

lymphoma equivalent of CLL

A

small lymphocytic lymphoma

65
Q

“starry-sky pattern” due to phagocytosis of apoptotic tumor cells

A

Burkitt

66
Q

associated with Sjogren, Hashimoto thyroiditis, and H. pylori

A

marginal cell MALToma

67
Q

cancer most commonly associated with a noninfectious fever

A

Hodgkin lymphoma

68
Q

sheets of lymphoid cells, with a “starry sky” appearance

A

Burkitt

69
Q

large B cells with bilobed nuclei and prominent Owl’s eye inclusions

A

Hodgkin lymphoma

70
Q

thyroid cells with optically clear nuclei

A

papillary carcinoma of thyroid

71
Q

anemia with hypersegmented neutrophils

A

megaloblastic anemia

72
Q

branching rods on oral infection

A

actinomyces israelii

73
Q

eczema + recurrent infections + thrombocytopenia

A

Wiskott-aldrich syndrome

74
Q

hemosiderinuria + thrombosis

A

paroxysmal nocturnal hemoglobinuria

75
Q

CD30 and CD15+ B cells

A

Reed-sternberg cells in Hodgkin’s lymphoma

76
Q

t(8:14)

A

Burkitt’s lymphoma

c-myc activation

77
Q

t(14:18)

A

diffuse large B cell and follicular lymphoma

bcl-2 activation

78
Q

t(11:14)

A

mantle cell lymphoma

cyclin D1 activation

79
Q

caused by HTLV-1

A

adult T-cell lymphoma

80
Q

t(9:22)

A

Philadelphia chromosome in CML

bcr-abl

81
Q

t(15:17)

A

AML (pro-myelocytic)

responsive to all-trans retinoic acid

82
Q

t(8:21)

A

AML in Down syndrome

83
Q

anemia, hypercalcemia, and bone pain has BM biopsy that shows plasma cells

A

multiple myeloma

84
Q

most common leukemia in children

A

ALL

85
Q

most common leukemia in adults in US

A

CLL

86
Q

characteristic Auer rods

A

AML

87
Q

myelodysplastic syndromes have a tendency to progress to

A

AML

88
Q

myeloproliferative disorders may progress to

A

AML

89
Q

more than 20% blasts in marrow

A

acute leukemia

90
Q

leukemia with more mature cells and <5% blasts

A

chronic leukemia

91
Q

PAS (+) acute leukemia

A

ALL

92
Q

commonly presents with bone pain

A

ALL

93
Q

numerous basophils, splenomegaly, and negative for leukocytic alkalnine phosphate (LAP)

A

CML

94
Q

always positive for philadelphia chromosome t(9:22)

A

CML

95
Q

acute leukemia positive for peroxidase

A

AML

96
Q

solid sheets of lymphoblasts in marrow

A

ALL

97
Q

PAS - acute leukemia

A

AML

98
Q

always associated with bcr-abl gene

A

CML

99
Q

“smudge” cells

A

CLL

100
Q

“punched out” lytic bone lesions

A

multiple myeloma

101
Q

RBCs clumped together like a stack of coins

A

multiple myeloma

102
Q

monoclonal antibody spike

A

multiple myeloma, monoclonal gammopathy of undetermined significance (MGUS), or Waldenstrom macroglobulinemia

103
Q

reddish-pink rods in cytoplasm of leukemic blasts

A

Auer rods in AML

104
Q

CD13/33 +

A

AML

105
Q

may progress to AML or ALL

A

CML

106
Q

95% have B cell markers

A

CLL

107
Q

associated with Down syndrome

A

ALL and AML

108
Q

associated with pancytopenia

A

Acute leukemia

109
Q

pancytopenia

A

anemia
bleeding tendency
infection

110
Q

multiple myeloma

A
think CRAB
hyperCalcemia
Renal insufficiency
Anemia
Back pain/lytic Bone lesions
111
Q

auer rods: what genetic mutation

A

t(15:17)

AML