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STEP1 2015 > HEME > Flashcards

HEME Flashcards

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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
megaloblastic anemia plus peripheral neuropathy
B12 deficiency
26
microcytic anemia + basophlic stippling
lead poisoning
27
microcytic anemia reversible with B6
sideroblastic anemia. p.391 | B6 is a cofactor for d-ALA synthase
28
HIV- positive patient with macrocytic anemia
zidovudine
29
drugs that cause sideroblastic anemia
seizure drugs INH chloramphenicol
30
drugs that cause nonmegaloblastic macrocytic anemia
5-FU zidovudine hydroxyurea
31
normocytic anemia with elevated creatinine
chronic kidney disease
32
uses for epo
cancer patients, ESRD, HIV infections
33
haptoglobin
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
34
warm agglutinins
``` 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 ```
35
cold agglutinins
extrinsic hemolytic normocytic anemia Cold weather is MMMiserable? igM Mycoplasma pneumonia infection infectious mononucleosis (EBV) CLL
36
Coombs test- direct
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
37
Coombs test- indirect
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
38
Ham's test
paroxysmal nocturnal hemoglobinuria
39
Heinz bodies
G6PD deficiency
40
vWF
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
lifespan of a PLT
8-10 days
42
What surface receptor is expressed after ADP is released from PLT, and is responsible for PLT aggregation
glycoprotein IIb/IIIa
43
NSAIDs prevent which platelet enzyme?
Thromboxane A2
44
what is the mechanism of streptokinase
converts plasminogen to plasmin | cleaves fibrin and breaks down clots
45
aspirin- mechanism
irreversibly inhibits COX-1
46
clopidogrel
blocks PLT ADP receptors
47
abciximab
monoclonal ab | binds glycoprotein IIb/IIIa on platelets
48
tirofiban
inhibits glycoprotein IIb/IIIa
49
ticlopidine
blocks ADP receptors
50
enoxaparin
LMWH, inhibits factor Xa
51
eptifibatide
glycoprotein IIb/IIIa inhibitor
52
HUNT for The Toilet Paper TTP
``` Hemolysis Uremia Neurological symptoms Thrombocytopenia Fever TTP ```
53
Nasty Fever Torched His Kidneys | TTP
``` Neurological symptoms Fever Thrombocytopenia Hemolysis Kidney failure ```
54
HUS
hemolysis renal insufficiency thrombocytopenia O157:H7 in children
55
anti GPIIB/IIIa ab
ITP. this is a chronic disease
56
ADAMTS-13 deficiency
TTP-HUS acute disease
57
GP2b/3a defect
Glanzmann's thrombasthenia chronic
58
vWF defect
vWF disease chronic
59
widespread activation of PLT and coag cascade
DIC acute
60
gp1b defect
Bernard-Soulier syndrome | chronic
61
causes of DIC
``` sepsis trauma obstetric complications acute pancreatitis malignancy nephrotic syndrome transfusions (STOP Making New Thrombi) ```
62
where does leukemia come from
bone marrow
63
where does lymphoma come from
lymph nodes
64
compare the age distribution of Hodgkin lymphoma to non-Hodgkin lymphoma
Hodgkin has a bimodal distribution peak age: 20 peak age: 65 NHL has a variable age distrubution
65
MC lymphoma in adults
diffuse large B cell lymphoma
66
MC lymphoma in children
lymphoblastic lymphoma
67
MC lymphoma in the US
diffuse large B cell lymphoma
68
Reed sternberg cells
Hodgkin lymphoma
69
particularly associated with EBV
Burkitt lymphoma, hodgkin lymphoma
70
associated with longterm celiac disease
intestinal T cell lymphoma
71
lymphoma equivalent of CLL
small lymphocytic lymphoma
72
starry sky pattern due to phagocytosis of apoptotic tumor cells
Burkitt lymphoma
73
associated with sjogren syndrome, hashimoto thyroiditis, h.pylori
NHL | marginal cell lymphoma -MALTOMA, an intestinal lymphoma
74
t(8;14)
Burkitt lymphoma
75
t(15;17)
M3 type of AML, treated with all- trans retinoic acid
76
t(14;18)
follicular lymphoma (BCL2 activation) always seen in CML Philadelphia CreaML cheese
77
t(9;22)
Philadelphia chromosome (Bcr-Abl constitutively active TKR oncogene) responds to imatinib
78
t(11;14)
Mantle cell lymphoma (cyclin D1 activation)
79
t(8;21)
AML | This is why Down syndrome patients have a higher incidence of AML
80
gene mutation commonly implicated in myeloproliferative disorders
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
Polycythemia vera
``` 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
Essential thrombocytosis
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
myelofibrosis
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
multiple myeloma versus Waldenstrom
MM- IgG Waldenstrom- IgM, no hypercalcemia, no renal involvement, no anemia, no lytic bone lesions, no back pain
85
Plasmacytoma
solid tumor of plasma cells - solitary plasmacytoma of bone - extramedullary plasmacytoma do not cause lytic bone lesions
86
Monoclonal gammopathy of undetermined significance (MGUS)
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
more than 20% blasts in marrow
acute leukemia
88
leukemia with more mature cells and
chronic leukemia
89
TdT(+) acute leukemia
ALL
90
commonly presents with bone pain
ALL
91
numerous basophils, splenomegaly, negative for leukocyte alkaline phosphatase (LAP)
CML
92
always positive for Philadelphia chromosome t(9;22)
CML
93
acute leukemia positive for peroxidase
AML (Auer rods)
94
solid sheets of lymphoblasts in marrow
ALL
95
Always associated with BCR- ABl gene
CML

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