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STEP1 2015 > HEME > Flashcards

HEME Flashcards

1
Q

acanthocyte

A

spur cell

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2
Q

degmacyte

A

bite cell

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3
Q

Echinocyte

A

burr cell

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4
Q

erythrocyte

A

RBC

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5
Q

Heinz body

A

oxidized hgb

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6
Q

Howell- Jolly body

A

nuclear remnant in patients with asplenia

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7
Q

Reticulocyte

A

immature RBC

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8
Q

Schistocyte

A

fragmented RBC

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9
Q

Spherocyte

A

spherical RBC

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10
Q

young liver synthesizes blood

A 
Yolk sac (3-8 weeks)
Liver (6wks- birth)
Spleen (10-28wks)- most important site of hematopoesis throughout fetal development
Bone marrow (18 wks to adult)

infancy/childhood: long/flat bones
sternum, pelvis, ribs, cranial bones, vertebrae, long bones of leg

adult: axial
vertebrae, sternum, ribs, pelvis

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11
Q

cytoplasmic ENZYME that is deficient in acute intermittent porphyria

A

uroporphyrinogen-1-synthase
aka porphobilinogen deaminase

which converts porphobilinogen to hydroxymethylbilane

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12
Q

Acute intermittent porphyria presentation

A

uroporphyrinogen-1-synthase
aka porphobilinogen deaminase

abdominal pain
"port wine" urine
polyneuropathy
psychological disturbances
precipitated by drugs
  • barbiturates
  • seizure drugs
  • rifampin
  • metoclopramide

Treat with glucose and heme, which inhibit delta- aminolevulinic acid synthase

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13
Q

delta- aminolevulinic acid synthase

A

rate limiting mitochondrial enzyme in heme synthesis

inhibited by glucose and heme

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14
Q

porphyria cutanea tarda

most common form of porphyria

A

primarily a skin problem
most common porphyria

presentation:
blistering of skin
photosensitivity
tea- colored urine
hypertrichosis
facial hyperpigmentation
associated with hep C and alcoholism

cytoplasmic enzyme: uroporphyrinogen decarboxylase

increased LFTs

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15
Q

mitochondrial enzyme inhibited in lead poisonint

A

ferrochelatase
ALA dehydratase
enzymes

protoporphytin and D-ALA accumulate in the blood

microcytic anemia (basophilic stippling), GI, colicky abdominal pain
HA, neurologic issues

encephalopathy
memory loss
delirium
mental deterioration
foot/wrist drop
lead lines (blue/black lines on the gums)
hyperlucent lines on metaphasis (of children)

renal failure

Tx: EDTA, succimer
dimercaprol instead of succimer if severe in a child

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16
Q

polycythemia (relative and absolute causes of erythrocytosis)

A 
relative= volume decreased
absolute= too many RBCs

Absolute can be appropriate (hypoxia) or inappropriate (ectopic epo or cancer)

polycythemia vera- monoclonal proliferation of RBCs

Chronic hypoxia
polymonary disease
sleep apnea
cyanotic heart disease
high altitudes

inappropriate increase in EPO
EPO- inducing tumor

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17
Q

EPO- producing tumors:

Potentially Really High HCT

A 
Pheochromocytoma
RCC
HCC
Hemangioblastoma
many trisomy 21 babies have polycythemia at birth
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18
Q

conditions that yield target cells

A

thalassemia
Hgb C disease
asplenia
liver disease

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19
Q

sideroblastic anemia

A

defective heme synthesis
ringed sideroblasts on bone marrow

causes:
hereditary form
chronic alcohol use
drugs (seizure drugs, isoniazid, chloramphenicol)

labs show increased iron and ferritin

Treat with B6

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20
Q

symptoms of folate deficiency

A

glossitis
increased homocysteine
normal MMA
NO Neurological deficits

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21
Q

symptoms of B12 deficiency

A

glossitis
neurologic deficits
increased homocysteine
increased MMA

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22
Q

microcytic anemia
swallowing difficulty
glossitis

A

Plummer-Vinson syndrome

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23
Q

microcytic anemia + >3.5% HbA2

A

beta thalassemia minor

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24
Q

megaloblastic anemia not correctable by B12 or folate

A

orotic aciduria

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25
Q

megaloblastic anemia plus peripheral neuropathy

A

B12 deficiency

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26
Q

microcytic anemia + basophlic stippling

A

lead poisoning

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27
Q

microcytic anemia reversible with B6

A

sideroblastic anemia. p.391

B6 is a cofactor for d-ALA synthase

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28
Q

HIV- positive patient with macrocytic anemia

A

zidovudine

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29
Q

drugs that cause sideroblastic anemia

A

seizure drugs
INH
chloramphenicol

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30
Q

drugs that cause nonmegaloblastic macrocytic anemia

A

5-FU
zidovudine
hydroxyurea

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31
Q

normocytic anemia with elevated creatinine

A

chronic kidney disease

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32
Q

uses for epo

A

cancer patients, ESRD, HIV infections

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33
Q

haptoglobin

A

binds free hgb in the blood (decreased in intravascular hemolysis). since haptoglobin does not bind up bilirubin with extravascular hemolysis you see unconjugated (indirect) bilirubin in extravascular hemolysis

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34
Q

warm agglutinins

A 
extrinsic hemolytic normocytic anemia
antibodies that react against RBC protein antigens at body temperature
IgG
seen in: 
1. EBV (warm or cold), HIV
2. lupus
3. malignancies including CLL
4. congenital immune abnormalities
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35
Q

cold agglutinins

A

extrinsic hemolytic normocytic anemia

Cold weather is MMMiserable?

igM
Mycoplasma pneumonia infection
infectious mononucleosis (EBV)
CLL

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36
Q

Coombs test- direct

A

red cell agglutination with addition of antihuman ab
because RBCs are coated with immunoglobulin or complement proteins

positive in autoimmune hemolytic anemia

  • hemolytic disease of the newborn
  • drug- induced autoimmune hemolytic anemia
  • hemolytic transfusion reactions
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37
Q

Coombs test- indirect

A

red cell agglutination with addition of patient’s serum to normal RBCs. This indicates that the serum contains anti-RBC surface Ig; RBCs agglutinate when Coombs reagent (antihuman abs) is added.

positive in autoimmune hemolytic anemia

  • screen blood before transfusion
  • screen maternal blood for anti-RBC fetal antibodies
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38
Q

Ham’s test

A

paroxysmal nocturnal hemoglobinuria

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39
Q

Heinz bodies

A

G6PD deficiency

40
Q

vWF

A

several subunits linked by disulfide bonds

synthesized by endothelial cells and megakaryocytes
major functions:
-complexes with and stabilizes factor VIII (deficiency leads to increased PTT)
-PLT adhesion to vessel wall, and other PLC (defect leads to increased bleeding time)

41
Q

lifespan of a PLT

A

8-10 days

42
Q

What surface receptor is expressed after ADP is released from PLT, and is responsible for PLT aggregation

A

glycoprotein IIb/IIIa

43
Q

NSAIDs prevent which platelet enzyme?

A

Thromboxane A2

44
Q

what is the mechanism of streptokinase

A

converts plasminogen to plasmin

cleaves fibrin and breaks down clots

45
Q

aspirin- mechanism

A

irreversibly inhibits COX-1

46
Q

clopidogrel

A

blocks PLT ADP receptors

47
Q

abciximab

A

monoclonal ab

binds glycoprotein IIb/IIIa on platelets

48
Q

tirofiban

A

inhibits glycoprotein IIb/IIIa

49
Q

ticlopidine

A

blocks ADP receptors

50
Q

enoxaparin

A

LMWH, inhibits factor Xa

51
Q

eptifibatide

A

glycoprotein IIb/IIIa inhibitor

52
Q

HUNT for The Toilet Paper

TTP

A 
Hemolysis
Uremia
Neurological symptoms
Thrombocytopenia
Fever
TTP
53
Q

Nasty Fever Torched His Kidneys

TTP

A 
Neurological symptoms
Fever
Thrombocytopenia
Hemolysis
Kidney failure
54
Q

HUS

A

hemolysis
renal insufficiency
thrombocytopenia

O157:H7 in children

55
Q

anti GPIIB/IIIa ab

A

ITP. this is a chronic disease

56
Q

ADAMTS-13 deficiency

A

TTP-HUS acute disease

57
Q

GP2b/3a defect

A

Glanzmann’s thrombasthenia chronic

58
Q

vWF defect

A

vWF disease chronic

59
Q

widespread activation of PLT and coag cascade

A

DIC acute

60
Q

gp1b defect

A

Bernard-Soulier syndrome

chronic

61
Q

causes of DIC

A 
sepsis
trauma
obstetric complications
acute pancreatitis
malignancy
nephrotic syndrome
transfusions (STOP Making New Thrombi)
62
Q

where does leukemia come from

A

bone marrow

63
Q

where does lymphoma come from

A

lymph nodes

64
Q

compare the age distribution of Hodgkin lymphoma to non-Hodgkin lymphoma

A

Hodgkin has a bimodal distribution
peak age: 20
peak age: 65

NHL has a variable age distrubution

65
Q

MC lymphoma in adults

A

diffuse large B cell lymphoma

66
Q

MC lymphoma in children

A

lymphoblastic lymphoma

67
Q

MC lymphoma in the US

A

diffuse large B cell lymphoma

68
Q

Reed sternberg cells

A

Hodgkin lymphoma

69
Q

particularly associated with EBV

A

Burkitt lymphoma, hodgkin lymphoma

70
Q

associated with longterm celiac disease

A

intestinal T cell lymphoma

71
Q

lymphoma equivalent of CLL

A

small lymphocytic lymphoma

72
Q

starry sky pattern due to phagocytosis of apoptotic tumor cells

A

Burkitt lymphoma

73
Q

associated with sjogren syndrome, hashimoto thyroiditis, h.pylori

A

NHL

marginal cell lymphoma -MALTOMA, an intestinal lymphoma

74
Q

t(8;14)

A

Burkitt lymphoma

75
Q

t(15;17)

A

M3 type of AML, treated with all- trans retinoic acid

76
Q

t(14;18)

A

follicular lymphoma (BCL2 activation) always seen in CML

Philadelphia CreaML cheese

77
Q

t(9;22)

A

Philadelphia chromosome (Bcr-Abl constitutively active TKR oncogene) responds to imatinib

78
Q

t(11;14)

A

Mantle cell lymphoma (cyclin D1 activation)

79
Q

t(8;21)

A

AML

This is why Down syndrome patients have a higher incidence of AML

80
Q

gene mutation commonly implicated in myeloproliferative disorders

A

JAK2 (Janus kinase 2- growth factor signaling, constitutive due to mutation).
plethora
pruritis after hot bath or shower
monoclonal proliferation of mature myeloid cells (may be one or several cell types)

81
Q

Polycythemia vera

A 
increased RBC synthesis with low EPO (consider JAK2 mutation) this is a myeloproliferative disorder
plethora
pruritis after hot bath or shower
splenomegaly
hyperviscosity of blood
HA
erythromelalgia
82
Q

Essential thrombocytosis

A

increased platelets with low thrombopoietin (consider JAK2 mutation) this is a myeloproliferative disorder)

thrombosis (too many platelets)
bleeding (the platelets the person has don’t work correctly)

83
Q

myelofibrosis

A

fibrosis and obliteration of marrow space

causes tear drop shaped RBCs

note that this is different from aplastic anemia (adipocytes fill the marrow space)

84
Q

multiple myeloma versus Waldenstrom

A

MM- IgG

Waldenstrom- IgM, no hypercalcemia, no renal involvement, no anemia, no lytic bone lesions, no back pain

85
Q

Plasmacytoma

A

solid tumor of plasma cells

  • solitary plasmacytoma of bone
  • extramedullary plasmacytoma

do not cause lytic bone lesions

86
Q

Monoclonal gammopathy of undetermined significance (MGUS)

A

monoclinal proliferation of plasma cells
asx
may lead to MM (1% per year)
no hypercalcemia, no renal involvement, no anemia, no lytic bone lesions or back pain

87
Q

more than 20% blasts in marrow

A

acute leukemia

88
Q

leukemia with more mature cells and

A

chronic leukemia

89
Q

TdT(+) acute leukemia

A

ALL

90
Q

commonly presents with bone pain

A

ALL

91
Q

numerous basophils, splenomegaly, negative for leukocyte alkaline phosphatase (LAP)

A

CML

92
Q

always positive for Philadelphia chromosome t(9;22)

A

CML

93
Q

acute leukemia positive for peroxidase

A

AML (Auer rods)

94
Q

solid sheets of lymphoblasts in marrow

A

ALL

95
Q

Always associated with BCR- ABl gene

A

CML

STEP1 2015 (30 decks)

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