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
diagnose beta-thalassemia minor
HbA2 on electrophoresis
26
lab findings of iron def:
decreased iron and ferritin, increased TIBC
27
what should be ruled out in 50 yo pt with new-onset iron deficiency anemia
colon cancer
28
microcytic anemia + swallowing difficulty + glossitis
Plummer-Vinson syndrome
29
microcytic anemia + >3.5% HbA2
B-thalassemia minor
30
megaloblastic anemia not correctable by B12 or folate
orotic aciduria
31
megaloblastic anemia along with peripheral neuropathy
B12 deficiency
32
microcytic anemia + basophilic stippling
lead poisoning
33
microcytic anemia reversible with B6
sideroblastic anemia
34
HIV-positive patient with macrocytic anemia
Zidovudine induced
35
Normocytic anemia and elevated creatinine
chronic renal disease
36
causes of hypochromic, microcytic anemias
iron deficiency, thalassemia, lead poisoning
37
hypersegmented neutrophils
megaloblastic anemia (B12 or folate)
38
skull x-ray shows a "hair-on-end" appearance
marrow hyperplasia
39
basophilic stippling of RBC
lead poisoning
40
findings associated with hereditary spherocytosis
anemia, jaundice, pigmented gallstones, splenomegaly, spherocytes + osmotic fragility test (-) Coombs test
41
Ham's test
Paroxysmal nocturnal hemoglobinuria | cells lyse when placed in acid (now use flow cytometry to look for no CD55/CD59)
42
Heinz bodies
G6PD deficiency
43
painful cyanosis of fingers and toes, with hemolytic anemia
cold autoimmune hemolytic anemia
44
red urine in the morning, and fragile RBC
paroxysmal nocturnal hemoglobinuria
45
basophilic nuclear remains in RBC
howell-jolly body
46
autosplenectomy
sickle cell disease
47
drug used to treat sickle cell disease
hydroxyurea
48
what does ADP and TxA2 do
ADP: induces GpIIb/IIIa expression on platelets TxA2: promotes crosslinking of GpIIb/IIIa by fribrinogen
49
cause of ITP
Ab against GpIIb/IIIa | lab shows increased megakaryocytes
50
Bernard-Soulier disease
defective GpIb
51
mom bleeds profusely from vagina and later gums after delivery
DIC
52
DIC
increased BT, PT, PTT, D-dimers decreased platelets and fibrinogen STOP Making Thrombi Sepsis, trauma, obstetrics, pancreatitis, malignancy, transfusions
53
antiplatelet antibodies
immune thrombocytopenia (ITP)
54
most common inherited bleeding disorder
von Willebrand disease
55
HUS-TTP
HUS triad: hemolysis (microangiopathic), renal insufficiency, thrombocytopenia TTP adds neurologic symptoms and fever
56
TTP
defect in ADAMTS 13 that causes failure to breakdown vWF multimers
57
Glanzmann's thrombasthenia
deficient GpIIb/IIIa | lab shows no platelet clumping
58
age distributions for Hodgkin and non-hodgkin lymphoma
Hodgkin peak at 20 and >65 | non-Hodgkin variable but 20-40 common
59
most common non-Hodgkin lymphoma in adults and children
adult: diffuse large B cell lymphoma children: lymphoblastic lymphoma
60
most common lymphoma in US
diffuse large B-cell
61
Reed-Sternberg cells
Hodgkin lymphoma
62
particularly associated with EBV
Burkitt, also Hodgkin lymphoma
63
associated with long-term celiac disease
intestinal
64
lymphoma equivalent of CLL
small lymphocytic lymphoma
65
"starry-sky pattern" due to phagocytosis of apoptotic tumor cells
Burkitt
66
associated with Sjogren, Hashimoto thyroiditis, and H. pylori
marginal cell MALToma
67
cancer most commonly associated with a noninfectious fever
Hodgkin lymphoma
68
sheets of lymphoid cells, with a "starry sky" appearance
Burkitt
69
large B cells with bilobed nuclei and prominent Owl's eye inclusions
Hodgkin lymphoma
70
thyroid cells with optically clear nuclei
papillary carcinoma of thyroid
71
anemia with hypersegmented neutrophils
megaloblastic anemia
72
branching rods on oral infection
actinomyces israelii
73
eczema + recurrent infections + thrombocytopenia
Wiskott-aldrich syndrome
74
hemosiderinuria + thrombosis
paroxysmal nocturnal hemoglobinuria
75
CD30 and CD15+ B cells
Reed-sternberg cells in Hodgkin's lymphoma
76
t(8:14)
Burkitt's lymphoma | c-myc activation
77
t(14:18)
diffuse large B cell and follicular lymphoma | bcl-2 activation
78
t(11:14)
mantle cell lymphoma | cyclin D1 activation
79
caused by HTLV-1
adult T-cell lymphoma
80
t(9:22)
Philadelphia chromosome in CML | bcr-abl
81
t(15:17)
AML (pro-myelocytic) | responsive to all-trans retinoic acid
82
t(8:21)
AML in Down syndrome
83
anemia, hypercalcemia, and bone pain has BM biopsy that shows plasma cells
multiple myeloma
84
most common leukemia in children
ALL
85
most common leukemia in adults in US
CLL
86
characteristic Auer rods
AML
87
myelodysplastic syndromes have a tendency to progress to
AML
88
myeloproliferative disorders may progress to
AML
89
more than 20% blasts in marrow
acute leukemia
90
leukemia with more mature cells and <5% blasts
chronic leukemia
91
PAS (+) acute leukemia
ALL
92
commonly presents with bone pain
ALL
93
numerous basophils, splenomegaly, and negative for leukocytic alkalnine phosphate (LAP)
CML
94
always positive for philadelphia chromosome t(9:22)
CML
95
acute leukemia positive for peroxidase
AML
96
solid sheets of lymphoblasts in marrow
ALL
97
PAS - acute leukemia
AML
98
always associated with bcr-abl gene
CML
99
"smudge" cells
CLL
100
"punched out" lytic bone lesions
multiple myeloma
101
RBCs clumped together like a stack of coins
multiple myeloma
102
monoclonal antibody spike
multiple myeloma, monoclonal gammopathy of undetermined significance (MGUS), or Waldenstrom macroglobulinemia
103
reddish-pink rods in cytoplasm of leukemic blasts
Auer rods in AML
104
CD13/33 +
AML
105
may progress to AML or ALL
CML
106
95% have B cell markers
CLL
107
associated with Down syndrome
ALL and AML
108
associated with pancytopenia
Acute leukemia
109
pancytopenia
anemia bleeding tendency infection
110
multiple myeloma
``` think CRAB hyperCalcemia Renal insufficiency Anemia Back pain/lytic Bone lesions ```
111
auer rods: what genetic mutation
t(15:17) | AML