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Step One R&M > Heme > Flashcards

Heme Flashcards

1
Q

5 causes of decreased sed rate

A

Polycythemia

Sickle cell anemia

CHF

Microcytosis

Hypofibringonemia

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1
Q

t(15:17)

A

PML (M3)

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2
Q

What binds LPS on gram - cell membrane?

A

CD14 receptor of macrophages

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2
Q

CD10+, TdT+

A

ALL

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2
Q

Auer rod

A

AML

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2
Q

Treatment of CML

A

Imatinib

(remember bcr-abl has tyrosine kinase activity)

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4
Q

Baby with failure to thrive, megaloblastic anemia not corrected by B12 or folate

A

Orotic aciduria

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4
Q

Mechanism of lymphopenia with GC use

A

Apoptosis

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4
Q

Neuropathy in adults and encephalopathy in kids

A

Lead poisoning

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5
Q

Defect in TTP

A

ADAMST13 = vWf MMP = long vWf = increased thrombosis and decreased platelet count

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5
Q

Macrophages present antigen via:

A

MHC class II

(to CD4+, think granulomas)

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5
Q

Cause of AIP

A

Defective porphobilinogen deaminase

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6
Q

Universal donor of blood

A

O-

(no antigens on RBCs)

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6
Q

What does hepcidin do?

A

Released from liver during inflammation, causing decreased iron release from intestine and macrophages –> ACD

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7
Q

Alpha granules of platelets

A

Fibrinogen, vWf

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8
Q

Cross-links fibrin

A

XIII

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8
Q

Orotic aciduria vs. OTC deficiency

A

Orotic aciduria - defective pyrimidine synthesis due to UMP synthase deficiency = high orotic acid, normal ammonia, megaloblastic anemia

OTC deficiency - defective urea cycle, high orotic acid, high ammonia, no megaloblastic anemia

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9
Q

Treatment of vW disease

A

DDAVP = increases stored vWf in endothelium

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10
Q

Indirect Coombs

A

Add normal RBCs to patients serum –> add anti-IgG –> agglutinate if patient’s serum contained IgG against RBCs (AHA)

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11
Q

Activates macrophages

A

IF-gamma

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11
Q

Cryoprecipitate contains:

A

vWf, factors VIII and XIII, fibrinogen

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12
Q

Which coagulation factor is carried by vWf?

A

VIII

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13
Q

Increased risk of parvo B19 crisis

A

SCD, beta thal major

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13
Q
A

PML (M3 AML)

(notice the fuck ton of auer rods)

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14
Q
A

Bite cell = G6PD deficiency

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15
Q
A

Sickle cell anemia

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15
Q

Precipitates AIP

A

Alcohol, starvation, phenytoin, griseofulvin, phenobarbital

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15
Q
A

Langerhans cell histiocytosis - lytic bone lesions in a kid

(S100+)

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16
Q

What do you give to increase clotting factors?

A

FFP

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17
Q

Two causes?

A

Hereditary spherocytosis

Autoimmune hemolysis

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18
Q

What is the “stippling” seen in lead poisoning and thalassemia?

A

rRNA (decreased degradation)

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19
Q

Treatment of PML?

A

ATRA - induces differentiation of myeloblasts

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20
Q

When does beta thal major manifest? Why?

A

6 months after birth - lose fetal Hb

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21
Q

Labs in ACD?

A

Decreased iron and TIBC, increased ferritin

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21
Q

Translocation of CML

A

bcr-abl, t(9:22)

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22
Q

Blood or tissue:

Monocyte, macrophage

A

Monocyte - macrophage precursor in blood

Macrophage - tissues

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22
Q

List the leukocytes from most to least abundant in normal blood

A

Neutrophil

Lymphocyte

Monocyte

Eosinophil

Basophil

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22
Q

bcl-2

A

Follicular lymphoma, anti-apoptotic oncogene

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23
Q

Medicinal cause of B12 deficiency

A

PPI’s

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23
Q

Diagnosis of vW disease

A

No agglutination of ristocetin cofactor assay

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24
Q

Universal recipient of blood

A

AB

(no antibodies)

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25
Q

Two pathways involved in subacute combined degeneration

A

Lateral CS tract

Dorsal columns

(Spasticity + defecits in vibration/proprioception; due to B12 deficiency)

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26
Q

Symptoms of AIP

A

Painful abdomen

Port wine-colored urine

Polyneuropathy

Psych disturbances

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27
Q
A

Beta thal major - marrow expansion

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29
Q

80% of circulating lymphocytes are:

A

T cells

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30
Q

Mast cells contain:

A

Heparin, eosinophil chemotactic factor, histamine

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30
Q

What causes Heinz bodies?

A

Oxidation of Hb sulfhydryl groups –> denatured Hb precipitates

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30
Q

CML vs. leukomoid reaction

A

Both have left shift

CML has low ALP, leukomoid reaction has high ALP

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31
Q

Hyperammonemia + orotic acidemia

A

OTC transcarbamylase deficiency - no urea cycle so extra ornithine converted into orotic acid

(X-linked urea cycle deficiency)

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31
Q

CD15+, CD30+

A

RS cell, Hodgkin’s lymphoma

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32
Q

Steps in primary hemostasis

A

Endothelial injury –> exposed collagen —> vWf binds collagen –> platelets binds vWf on endothelium via GpIb –> platelets release ADP –> better adherence to endothelium + upregulation of GpIIb/IIIa –> platelets bind fibrinogen

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32
Q

RBC pathology seen in megaloblastic anemia

A

Macro-ovalocyte

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32
Q

Treatment for sideroblastic anemia?

A

B6 = cofactor for ALA synthase

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32
Q

S100+

A

Langerhans cell histiocytosis

Melanoma

Schwannoma

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33
Q

What causes Howell-Jolly bodies?

A

Nuclear remnants in RBC that cannot be cleared by macrophages due to asplenia/hyposplenia

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34
Q

Dense granules of platelets

A

ATP, calcium

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35
Q

4 infections that can cause aplastic anemia

A

Parvo

HIV

HVC

EBV

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36
Q

Most common cause of death in SCD adults

A

Acute chest syndrome

(vaso-occlusive disease of pulmonary vasculature)

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37
Q

Cyclin D1

A

Mantle cell lymphoma

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38
Q
A

Pseudo-Pelger-Huetz anomaly

(bilobed PMN after chemo)

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39
Q

t(8:14)

A

Burkitt lymphoma

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39
Q

TdT+

A

ALL

(marker of pre-B and pre-T cell)

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40
Q

Converts plasminogen to plasmin

A

Kallikrein and tPA

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41
Q

Treat with glucose

A

AIP (inhibits ALA synthase)

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42
Q

Diffuse large B-cell lymphoma translocation

A

t(14:18)

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43
Q

What is a “blast crisis”?

A

Transformation of CML into AML or ALL

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44
Q

JAK2 mutation

A

Polycthemia vera

Essential thrombocytosis

Myelofibrosis

(myeloproliferative D/O’s other than CML)

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45
Q
A

Heinz bodies = G6PD deficiency

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46
Q
A

Acanthocyte

Liver disease, abetalipoproteinemia

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46
Q

How does adult T cell lymphoma present?

A

Lytic bone lesions and hypercalcemia

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47
Q

Lytic bones lesions and skin rash in a kid

A

Langerhands cell histiocytosis

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48
Q

CD14+

A

Macrophage

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48
Q

Mantle cell lymphoma marker

A

CD5+

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49
Q

Isolated basophilia

A

CML

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51
Q

Azurophilic granules of PMNs are actually:

A

Lysosomes

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52
Q

Causes?

A

HbC

Asplenia

Liver disease

Thalassemia

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52
Q
A

CLL

(CD5+, CD20+)

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53
Q
A

Heinz bodies = G6PD deficiency

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54
Q

HbC disease

A

Glutamic acid –> lysine at position 6 of beta gene

Target cell

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56
Q

GpIb

A

vWf receptor

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57
Q

Co-stimulatory signal for T cell activation

A

CD28

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57
Q

Very low leukocyte alk phos

A

CML (vs. leukomoid reaction)

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58
Q

Defect in ITP

A

Autoantibodies against GpIIb/IIIa

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60
Q
A

Hereditary elliptocytosis

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61
Q

Cause of orotic aciduria?

A

UMP synthase deficiency = can’t convert orotic acid to UMP in pydrimidine synthesis

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63
Q

Markers of ALL

A

TdT+, CD10+

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64
Q

Heparin, histamine, leukotrienes

A

Basophils

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65
Q

Increased HbA2

A

Beta thal minor

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66
Q

What causes the appearance of sideroblastics?

A

Too much iron in mitochondria

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67
Q
A

Hairy cell leumia, TRAP stain

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69
Q

Decreased haptoglobin

A

IV hemolysis

(Binds up all the free Hb and takes it to the RES, so reduced numbers in blood)

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70
Q

Causes of warm agglutinin AHA

A

Alpha-methyldopa

CLL

SLE

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72
Q

Defect in Bernard-Solier

A

GpIb

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73
Q
A

Howell Jolly bodies (asplenia)

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74
Q
A

ALL

(TdT+, CD10+)

74
Q

How does Langerhans cell histiocytosis present?

A

Kid with rash + lytic bone lesions or recurrent otitis media with mastoid mass

76
Q

Direct Combs

A

Add anti-IgG to patient’s blood –> agglutinate if IgG attached to RBCs (AHA)

76
Q

Manifestation of essential thrombocytosis

A

Bleeding, thrombosis (increased number of ABNORMAL platelets)

78
Q

Small, more numerous granules in PMNs contain:

A

Lactoferrin, lysozyme, alk phos, collagenase

80
Q

Cell of type I hypersensitivity

81
Q

How does CML differ from other myeloproliferative disorders?

A

No JAK2 mutation

+ t(9:22)

82
Q

CD21+

A

B cell, binds C3d

82
Q

Dry tap on BM aspiration

A

Myelofibrosis

Hairy cell leukemia

83
Q

Follicular lymphoma translocation

A

t(14:18), bcl-2

84
Q

Normal methymalonic acid but increased homocystine

A

Folate deficiency

85
Q
A

Asplenia

(notice Howell Jolly bodies + target cell)

86
Q

Problem in Factor V leiden?

A

Factor V is resistant to degradation by protein C = hypercoagulated

87
Q

Markers of mature B cell

A

CD19, CD20, CD21

88
Q

Normal platelet count and PT/PTT but prolonged bleeding time

A

Glanzmann thromboasthenia (no GpIIb/IIIa)

89
Q

Asymptomatic precursor to MM

90
Q

Large, less numerous, azurophilic PMN granules contain:

A

beta-glucuronidase, MPO, acid phosphatase, proteinase

91
Q

Leukemia associated with AHA

A

CLL

(warm or cold agglutinins)

92
Q

CD5+

A

Mantle cell lymphoma (t11:14)

CLL

93
Q

FoxP3

95
Q

Defective ALA synthase vs. dehydratase

A

Synthase = sideroblastic anemia

Dehydratase = lead poisoning (+ defective ferrochelatase)

95
Q

Two infections that cause hemolysis

A

Malaria

Babesia

96
Q

M spike = mostly IgG with some IgA

97
Q

Stores fibrinogen

A

Alpha granule of platelet

98
Q

Converts HMWK –> Bradykinin

A

Kallikrein

99
Q

Anemia + neuro symptoms

A

Lead poisoning

B12 deficiency

TTP

100
Q

Who gets Mantle cell lymphoma?

A

Older males

101
Q

HbH

A

ß4 - seen in three allele deletion in alpha thal

102
Q
A

Langerhans cell histiocytosis (Birbeck granules)

103
Q

CD20+

A

B cell

(think CLL)

105
Q

Hemolytic anemia in a newborn

A

Pyruvate kinase deficiency

(No glycolysis –> RBCs lyse)

106
Q

One symptom shared by SCD and sickle cell trait

A

Renal papillary necrosis

(O2 tension in renal papilla low enough to induce sickling in SC trait)

108
Q

vW disease vs. hemophilia A or B

A

vW disease will prolong bleeding time, while hemophilia is purely a clotting factor problem (increased PTT)

109
Q

Prolong PTT and bleeding time with normal platelet count and PT

A

vW disease

110
Q

Basophi granules

A

Heparin, histamine, leukotrienes

112
Q

Oncogene of Burkitt lymphoma

A

c-myc (transcription factor)

114
Q

RBC, WBC, platelet:

PV

Essential thrombocytosis

Myelofibrosis

CML

A

PV - everything increased

Essential thrombocytosis - just platelets increased

Myelofibrosis - RBCs decreased, others variable

CML - decreased RBCs, increased WBC and platelets

115
Q

Better prognosis of ALL

116
Q

Two RBC pathologies seen in lead poisoning

A

Basophilic stippling + ringed sideroblasts

118
Q

t(9:22)

A

Philadelphia chromosome - CML - BCR-ABL (tyrosine kinase)

119
Q

G6PD deficiency cells

A

Bite cells + Heinz bodies

120
Q

Predisposing factors include prior chemo, radiation, myeloprolierative disorder

121
Q

Follicular lymphoma oncogen

122
Q

Protein C and S inactivate:

A

V and VIII

122
Q

What is the function of haptoglobin?

A

Binds free hemoglobin and takes it to the spleen

123
Q

During primary hemostasis, ADP is release from:

A

Platelet alpha granules to upregulate expression on GpIIb/IIIa

123
Q

Pregnancy and OCPs increase production of (regarding iron):

A

Transferrin, so increased TIBC

125
Q
A

Beta thal major or SCD - marrow expansion

126
Q

+ Coombs means:

A

Autoimmune (must have had IgG on RBCs)

127
Q

Origin of hairy cell leukemia

128
Q

Why do neonates get IM vitamin K when born?

A

Lack enteric bacteria which produce vitamin K

130
Q

Two jobs of kallikrein

A
  1. HMWK –> BK
  2. Plasminogen –> plasmin
132
Q

6 drugs that can cause aplastic anemia

A

PTU

Methimazole

NSAIDs

Chloramphenicol

Benzene

Carbamazepine

133
Q

CD3+

A

Mature T cell

(part of T cell receptor complex)

135
Q

ALL translocation

A

t(12:21), associated with better prognosis

136
Q

Where does vWf come from?

A

Platelets and endothelial cells

137
Q

Peroxidase + cytoplasmic inclusion

A

Auer rod = AML

139
Q

No reticulocytes despite anemia

A

Aplastic anemia

140
Q

Normal PT/PTT but no increase in PTT after heparin

A

ATIII deficiency

(congenital, nephrotic syndrome, renal failure)

141
Q

bcr-abl

A

CML (tyrosine kinase)

142
Q

Treatment of beta thal

A

Blood transfusions

143
Q

Two RBC pathologies in liver disease?

A

Acanthocytes

Target cells

144
Q

Markers of Hodgkin’s lymphoma

A

CD15+, CD30+

145
Q

Proteinases, acid phosphatase, beta-glucuronidase, MPO

A

Azurophilic (lysosome) granules of PMN

146
Q

Labs?

A

Increased iron and ferritin, normal TIBC

(Ringed sideroblasts - too much iron in mitochondria)

147
Q

Anemia + bone deformations + hepatsplenomegaly

A

Beta thalassemia

(Bone malformations and hepatosplenomegaly due to expanded marrow and extramedullary hematopoeisis, respectively)

149
Q

Painless waxing and waning lymphadenopathy

A

Follicular lymphoma

150
Q

GpIIb/IIIa

A

Binds fibrinogen

151
Q

Origin of RS cell

A

B cell (CD15+ and CD30+)

152
Q

CD19+

153
Q
A

Myelofibrosis

(tear drop cells)

155
Q

Treatment for hereditary spherocytosis

A

Splenectomy

(macrophages in spleen keep removing defective RBCs)

157
Q

Converts fibringogen to fibrin

158
Q

Myeloproliferative disorders can transform into:

160
Q

B thal minor

A

Underproduced beta chain

Generally asymptomatic

HbA2 > 3.5%

161
Q
A

Sideroblastic anemia - too much iron in RBC

162
Q

Increased methylmalonic acid + homocystine

A

B12 deficiency

163
Q

Labs?

A

Decreased iron and ferritin, increased TIBC

164
Q

Mantle cell lymphoma translocation

A

t(11:14); cyclin D1 to Ig

165
Q

Drugs that can precipitate oxidant stress in G6PD deficiency

A

Sulfas

Primaquine

Dapsone

INH

Aspirin

Ibuprofen

Nitrofurantoin

167
Q

Why does orotic aciduria cause macrocytic anemia?

A

Defective pyrimidine synthesis = no DNA = megaloblastic RBCs

169
Q

3 forms of alpha thalassemia

A
  1. Hb Barts - no alpha allele, gamma4, hyrdops fetalis
  2. HbH - 3 defective alpha alleles, ß4
  3. 1-2 alpha allele deletions, clinically insignificant
170
Q

Enlarged megakaryocytes

A

Essential thrombocytosis, ITP

171
Q

CD4+ skin lesions

A

Mycosis fungoides

172
Q

Causes of sideroblastic anemia

A

ALA synthase deficiency

Copper deficiency

Lead poisoning

Alcohol

B6 deficiency

INH

Myelodysplastic syndromes

173
Q

Least abundant leukocyte in normal blood

174
Q

Where in the anemia classification scheme do thalassemias fall?

A

Microcytic

174
Q

What do you give to hemophilia patient?

A

Recombinant factor VIII or cryoprecipitate

175
Q

Causes of cold agglutinin AHA

A

Mycoplasma

Mono

CLL

176
Q

Protected against P. falciparum

A

Sickle cell TRAIT

177
Q

MC non-Hodgkin lymphoma in adults

A

Diffuse large B-cell lymphoma (t14:18)

178
Q

c-myc

A

Burkitt lymphoma (transcription factor)

179
Q

Smudge cells

180
Q

Treat with heme

A

AIP (inhibits ALA synthase)

181
Q

Isolated basophilia

A

CML!!!!!!!!

183
Q

How do you diagnose monoclonal gammopathy of undertermined significance?

A

M spike with < 3 g/dl serum monoclonal protein

184
Q

Bet thal major

A

Absent beta chain

Extramedullary hematopoeisis (hepatosplenomegaly)

Skull deformations (medullary expansion)

186
Q

Mechanism of eosinopenia with GC use

A

Sequesters eosinophils in lymph nodes

187
Q

Decreased platelet count + increased bleeding time

A

Bernard-Soulier (no GpIb)

ITP (Ab against GpIIb/IIIa)

TTP (no ADAMTS13 = vWf MMP)

189
Q

Decreased platelet count + prolonged BT and PT/PTT

190
Q

Wrist and foot drop in kid

A

Lead poisoning

(give SUCCIMER)

191
Q

Infectious cause of megaloblastic anemia?

A

Diphyllobothrium latum (fish tapeworm) = B12 deficiency

192
Q

Leukemia most associated with DIC

A

Promyelocytic leukmia (AML M3)

(can be induced by chemo –> due to release of Auer rods)

194
Q

Recurrent otitis media in kid (if cancer)

A

Langerhans cell histiocytosis

(ineffective Langerhans cells can’t present to T cells)

195
Q

Burkitt lymphoma translocation

196
Q

Heparin, histamine, eosinophil chemotactic factor

A

Mast cells

198
Q

CD28+

A

Co-stimulatory signal for T cell activation

199
Q

Eosinophils contain:

A

Arylsulfatase, heparinase

200
Q

Hemolytic anemia + venous thrombis + pancytopenia

A

PNH (no GPI anchor for DAF)

201
Q

t(11:14)

A

Mantle cell lymphoma; cyclin D1 to Ig

202
Q

How do nephrotic syndrome and renal failure cause hypercoagulability?

A

Loss of ATIII in urine = increased factors II and X

203
Q

Diagnosis of PNH

A

CD55/59 - on flow cytometry

205
Q

Treatment of hairy cell leukemia

A

Cladribine (2-CA)

Adenosine analog resistant to ADA

206
Q

3 functions of BK

A
  1. Vasodilation
  2. Pain
  3. Increase permeability
207
Q

CD10+

A

ALL

(Pre-B cell marker)

208
Q

Labs in hemochromatosis

A

Increased iron and ferritin and % transferrin sat

Decreased transferrin/TIBC

209
Q

Point mutation in SCD

A

Glutamic acid –> valine at position 6 of beta gene

210
Q

Two RBC pathologies in thalassemia?

A

Basophilic stippling

Target cells

211
Q

Causes?

A

DIC

TTP/HUS

Prosthetic valve

212
Q

Marker of macrophage

A

CD14 (binds LPS)

213
Q

Skin necrosis after warfarin administration

A

Protein C deficiency = can’t break down V or VIII and warfarin only breaks down II, VII, IX, and X

215
Q

What substrate is present in the urine of an AIP patient during an attack?

A

Coporphobilinogen

216
Q

Best prognosis for Hodgkin’s?

A

Lymphocyte rich form

217
Q

RBC pathology in abetalipoproteinemia

A

Acanthryocyte (spiny cell)

218
Q

CD25+

219
Q

Alk phos, collagenase, lactoferrin, lysozyme

A

PMN small, numerous granules

220
Q

Triad of PNH

A

Hemolytic anemia + venous thrombus + pancytopenia

221
Q

Defect in Glanzmann thromboasthenia

A

GpIIb/IIIa receptor

222
Q

DIC vs. TTP

A

DIC = low platelet count + prolonged BT and PT/PTT

TTP = only platelets are jacked up, so PT/PTT are normal while platelet count is low and BT is prolonged

223
Q

Pseudo-Pelger-Huet anomaly

A

Bilobed neutrophils (hyposegmented) typically following chemo

224
Q

Two jobs of plasmin

A
  1. Degrades fibrin
  2. Activates complement (C3 –> C3a)
225
Q

What causes bite cells?

A

Removal of precipitated Hb by phagocytes

226
Q

Who gets adult T cell lymphoma?

A

IVDA (HTLV-1)

227
Q

Urobilinogen vs. unconjugated bilirubin in urin

A

Urobilinogen = IV hemolysis

Unconjugated bilirubin = EV hemolysis (also jaundiced)

229
Q

Mycosis fungoides marker

230
Q

1-2 alpha chain allele deletions

A

No clincally significant anemia

231
Q

M spike = IgM

A

Waldenstrom’s macroglobulinemia (hyperviscosity syndrome)

232
Q

Treatment of AIP

A

Glucose and heme, which inhibit ALA synthase

233
Q

t(14:18)

A

Diffuse large B-cell lymphoma

Follicular lymphoma, bcl-2

234
Q

Unique to Tregs

A

FoxP3, CD25+

235
Q

Hb barts

A

Gamma4 - seen in 4 allele deletion of alpha thal - incompatible with life (hydrops fetalis)

Step One R&M (38 decks)

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