Heme Flashcards Preview

Step One R&M > Heme > Flashcards

Flashcards in Heme Deck (235):
1

5 causes of decreased sed rate

Polycythemia

Sickle cell anemia

CHF

Microcytosis

Hypofibringonemia

1

t(15:17)

PML (M3)

2

What binds LPS on gram - cell membrane?

CD14 receptor of macrophages

2

CD10+, TdT+

ALL

2

Auer rod

AML

2

Treatment of CML

Imatinib

 

(remember bcr-abl has tyrosine kinase activity)

4

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

Orotic aciduria

4

Mechanism of lymphopenia with GC use

Apoptosis

4

Neuropathy in adults and encephalopathy in kids

Lead poisoning

5

Defect in TTP

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

5

Macrophages present antigen via:

MHC class II 

 

(to CD4+, think granulomas)

5

Cause of AIP

Defective porphobilinogen deaminase

6

Universal donor of blood

O-

(no antigens on RBCs)

6

What does hepcidin do?

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

7

Alpha granules of platelets

Fibrinogen, vWf

8

Cross-links fibrin

XIII

8

Orotic aciduria vs. OTC deficiency

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

9

Treatment of vW disease

DDAVP = increases stored vWf in endothelium

10

Indirect Coombs

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

11

Activates macrophages

IF-gamma

11

Cryoprecipitate contains:

vWf, factors VIII and XIII, fibrinogen

12

Which coagulation factor is carried by vWf?

VIII

13

Increased risk of parvo B19 crisis

SCD, beta thal major

13

PML (M3 AML)

 

 

(notice the fuck ton of auer rods)

14

Bite cell = G6PD deficiency

15

Sickle cell anemia

15

Precipitates AIP

Alcohol, starvation, phenytoin, griseofulvin, phenobarbital

15

Langerhans cell histiocytosis - lytic bone lesions in a kid

 

(S100+)

16

What do you give to increase clotting factors?

FFP

17

Two causes?

Hereditary spherocytosis

Autoimmune hemolysis

18

What is the "stippling" seen in lead poisoning and thalassemia?

rRNA (decreased degradation)

19

Treatment of PML?

ATRA - induces differentiation of myeloblasts

20

When does beta thal major manifest? Why?

6 months after birth - lose fetal Hb

21

Labs in ACD?

Decreased iron and TIBC, increased ferritin

21

Translocation of CML

bcr-abl, t(9:22)

22

Blood or tissue:

Monocyte, macrophage

Monocyte - macrophage precursor in blood

Macrophage - tissues

22

List the leukocytes from most to least abundant in normal blood

Neutrophil

Lymphocyte

Monocyte

Eosinophil

Basophil

22

bcl-2

Follicular lymphoma, anti-apoptotic oncogene

23

Medicinal cause of B12 deficiency

PPI's

23

Diagnosis of vW disease

No agglutination of ristocetin cofactor assay

24

Universal recipient of blood

AB

(no antibodies)

25

Two pathways involved in subacute combined degeneration

Lateral CS tract

Dorsal columns

 

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

26

Symptoms of AIP

Painful abdomen

Port wine-colored urine

Polyneuropathy

Psych disturbances

27

Beta thal major - marrow expansion

29

80% of circulating lymphocytes are:

T cells

30

Mast cells contain:

Heparin, eosinophil chemotactic factor, histamine

30

What causes Heinz bodies?

Oxidation of Hb sulfhydryl groups --> denatured Hb precipitates

30

CML vs. leukomoid reaction

Both have left shift

 

CML has low ALP, leukomoid reaction has high ALP

31

Hyperammonemia + orotic acidemia

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

 

(X-linked urea cycle deficiency)

31

CD15+, CD30+

RS cell, Hodgkin's lymphoma

32

Steps in primary hemostasis

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

32

RBC pathology seen in megaloblastic anemia

Macro-ovalocyte

32

Treatment for sideroblastic anemia?

B6 = cofactor for ALA synthase

32

S100+

Langerhans cell histiocytosis

Melanoma

Schwannoma

33

What causes Howell-Jolly bodies?

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

34

Dense granules of platelets

ATP, calcium

35

4 infections that can cause aplastic anemia

Parvo

HIV

HVC

EBV

36

Most common cause of death in SCD adults

Acute chest syndrome

 

(vaso-occlusive disease of pulmonary vasculature)

37

Cyclin D1

Mantle cell lymphoma

38

Pseudo-Pelger-Huetz anomaly

 

(bilobed PMN after chemo)

39

t(8:14)

Burkitt lymphoma

39

TdT+

ALL

 

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

40

Converts plasminogen to plasmin

Kallikrein and tPA

41

Treat with glucose

AIP (inhibits ALA synthase)

42

Diffuse large B-cell lymphoma translocation

t(14:18)

43

What is a "blast crisis"?

Transformation of CML into AML or ALL

44

JAK2 mutation

Polycthemia vera

Essential thrombocytosis

Myelofibrosis

 

(myeloproliferative D/O's other than CML)

45

Heinz bodies = G6PD deficiency

46

Acanthocyte

 

Liver disease, abetalipoproteinemia

46

How does adult T cell lymphoma present?

Lytic bone lesions and hypercalcemia

47

Lytic bones lesions and skin rash in a kid

Langerhands cell histiocytosis

48

CD14+

Macrophage

48

Mantle cell lymphoma marker

CD5+

49

Isolated basophilia

CML

51

Azurophilic granules of PMNs are actually:

Lysosomes

52

Causes?

HbC

Asplenia

Liver disease

Thalassemia

52

CLL

 

(CD5+, CD20+)

53

Heinz bodies = G6PD deficiency

54

HbC disease

Glutamic acid --> lysine at position 6 of beta gene

 

Target cell

56

GpIb

vWf receptor

57

Co-stimulatory signal for T cell activation

CD28

57

Very low leukocyte alk phos

CML (vs. leukomoid reaction)

58

Defect in ITP

Autoantibodies against GpIIb/IIIa

60

Hereditary elliptocytosis

61

Cause of orotic aciduria?

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

63

Markers of ALL

TdT+, CD10+

64

Heparin, histamine, leukotrienes

Basophils

65

Increased HbA2

Beta thal minor

66

What causes the appearance of sideroblastics?

Too much iron in mitochondria

67

Hairy cell leumia, TRAP stain

69

Decreased haptoglobin

IV hemolysis

 

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

70

Causes of warm agglutinin AHA

Alpha-methyldopa

CLL

SLE

72

Defect in Bernard-Solier

GpIb

73

Howell Jolly bodies (asplenia)

74

ALL

 

(TdT+, CD10+)

74

How does Langerhans cell histiocytosis present?

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

76

Direct Combs

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

76

Manifestation of essential thrombocytosis

Bleeding, thrombosis (increased number of ABNORMAL platelets)

78

Small, more numerous granules in PMNs contain:

Lactoferrin, lysozyme, alk phos, collagenase

80

Cell of type I hypersensitivity

Mast cell

81

How does CML differ from other myeloproliferative disorders?

No JAK2 mutation

+ t(9:22)

82

CD21+

B cell, binds C3d

82

Dry tap on BM aspiration

Myelofibrosis

Hairy cell leukemia

83

Follicular lymphoma translocation

t(14:18), bcl-2

84

Normal methymalonic acid but increased homocystine

Folate deficiency

85

Asplenia

 

(notice Howell Jolly bodies + target cell)

86

Problem in Factor V leiden?

Factor V is resistant to degradation by protein C = hypercoagulated

87

Markers of mature B cell

CD19, CD20, CD21

88

Normal platelet count and PT/PTT but prolonged bleeding time

Glanzmann thromboasthenia (no GpIIb/IIIa)

89

Asymptomatic precursor to MM

MGUS

90

Large, less numerous, azurophilic PMN granules contain:

beta-glucuronidase, MPO, acid phosphatase, proteinase

91

Leukemia associated with AHA

CLL

 

(warm or cold agglutinins)

92

CD5+

Mantle cell lymphoma (t11:14)

CLL

93

FoxP3

Treg

95

Defective ALA synthase vs. dehydratase

Synthase = sideroblastic anemia

 

Dehydratase = lead poisoning (+ defective ferrochelatase)

95

Two infections that cause hemolysis

Malaria

Babesia

96

M spike = mostly IgG with some IgA

MM

97

Stores fibrinogen

Alpha granule of platelet

98

Converts HMWK --> Bradykinin

Kallikrein

99

Anemia + neuro symptoms

Lead poisoning

B12 deficiency

TTP

100

Who gets Mantle cell lymphoma?

Older males

101

HbH

ß4 - seen in three allele deletion in alpha thal

102

Langerhans cell histiocytosis (Birbeck granules)

103

CD20+

B cell

 

(think CLL)

105

Hemolytic anemia in a newborn

Pyruvate kinase deficiency

 

(No glycolysis --> RBCs lyse)

106

One symptom shared by SCD and sickle cell trait

Renal papillary necrosis

 

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

108

vW disease vs. hemophilia A or B

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

109

Prolong PTT and bleeding time with normal platelet count and PT

vW disease

110

Basophi granules

Heparin, histamine, leukotrienes

112

Oncogene of Burkitt lymphoma

c-myc (transcription factor)

114

RBC, WBC, platelet:

PV

Essential thrombocytosis

Myelofibrosis

CML

PV - everything increased

Essential thrombocytosis - just platelets increased

Myelofibrosis - RBCs decreased, others variable

CML - decreased RBCs, increased WBC and platelets

115

Better prognosis of ALL

t(12:21)

116

Two RBC pathologies seen in lead poisoning

Basophilic stippling + ringed sideroblasts

118

t(9:22)

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

119

G6PD deficiency cells

Bite cells + Heinz bodies

120

Predisposing factors include prior chemo, radiation, myeloprolierative disorder

AML

121

Follicular lymphoma oncogen

bcl-2

122

Protein C and S inactivate:

V and VIII

122

What is the function of haptoglobin?

Binds free hemoglobin and takes it to the spleen

123

During primary hemostasis, ADP is release from:

Platelet alpha granules to upregulate expression on GpIIb/IIIa

123

Pregnancy and OCPs increase production of (regarding iron):

Transferrin, so increased TIBC

125

Beta thal major or SCD - marrow expansion

126

+ Coombs means:

Autoimmune (must have had IgG on RBCs)

127

Origin of hairy cell leukemia

B cell

128

Why do neonates get IM vitamin K when born?

Lack enteric bacteria which produce vitamin K

130

Two jobs of kallikrein

1. HMWK --> BK

 

2. Plasminogen --> plasmin

132

6 drugs that can cause aplastic anemia

PTU

Methimazole

NSAIDs

Chloramphenicol

Benzene

Carbamazepine

133

CD3+

Mature T cell

 

(part of T cell receptor complex)

135

ALL translocation

t(12:21), associated with better prognosis

136

Where does vWf come from?

Platelets and endothelial cells

137

Peroxidase + cytoplasmic inclusion

Auer rod = AML

139

No reticulocytes despite anemia

Aplastic anemia

140

Normal PT/PTT but no increase in PTT after heparin

ATIII deficiency 

 

(congenital, nephrotic syndrome, renal failure)

141

bcr-abl

CML (tyrosine kinase)

142

Treatment of beta thal

Blood transfusions

143

Two RBC pathologies in liver disease?

Acanthocytes

Target cells

144

Markers of Hodgkin's lymphoma

CD15+, CD30+

145

Proteinases, acid phosphatase, beta-glucuronidase, MPO

Azurophilic (lysosome) granules of PMN

146

Labs?

Increased iron and ferritin, normal TIBC

 

 

(Ringed sideroblasts - too much iron in mitochondria)

147

Anemia + bone deformations + hepatsplenomegaly

Beta thalassemia

 

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

149

Painless waxing and waning lymphadenopathy

Follicular lymphoma

150

GpIIb/IIIa

Binds fibrinogen

 

151

Origin of RS cell

B cell (CD15+ and CD30+)

152

CD19+

B cell

153

Myelofibrosis

 

(tear drop cells)

155

Treatment for hereditary spherocytosis

Splenectomy

 

(macrophages in spleen keep removing defective RBCs)

157

Converts fibringogen to fibrin

Thrombin

158

Myeloproliferative disorders can transform into:

AML

160

B thal minor

Underproduced beta chain

Generally asymptomatic

HbA2 > 3.5%

161

Sideroblastic anemia - too much iron in RBC

162

Increased methylmalonic acid + homocystine

B12 deficiency

163

Labs?

Decreased iron and ferritin, increased TIBC

164

Mantle cell lymphoma translocation

t(11:14); cyclin D1 to Ig

165

Drugs that can precipitate oxidant stress in G6PD deficiency

Sulfas

Primaquine

Dapsone

INH

Aspirin

Ibuprofen

Nitrofurantoin

167

Why does orotic aciduria cause macrocytic anemia?

Defective pyrimidine synthesis = no DNA = megaloblastic RBCs

169

3 forms of alpha thalassemia

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

Enlarged megakaryocytes

Essential thrombocytosis, ITP

171

CD4+ skin lesions

Mycosis fungoides

172

Causes of sideroblastic anemia

ALA synthase deficiency

Copper deficiency

Lead poisoning

Alcohol

B6 deficiency

INH

Myelodysplastic syndromes

173

Least abundant leukocyte in normal blood

Basophil

174

Where in the anemia classification scheme do thalassemias fall?

Microcytic

174

What do you give to hemophilia patient?

Recombinant factor VIII or cryoprecipitate

175

Causes of cold agglutinin AHA

Mycoplasma

Mono

CLL

176

Protected against P. falciparum

Sickle cell TRAIT

177

MC non-Hodgkin lymphoma in adults

Diffuse large B-cell lymphoma (t14:18)

178

c-myc

Burkitt lymphoma (transcription factor)

179

Smudge cells

CLL

180

Treat with heme

AIP (inhibits ALA synthase)

181

Isolated basophilia

CML!!!!!!!!

183

How do you diagnose monoclonal gammopathy of undertermined significance?

M spike with < 3 g/dl serum monoclonal protein

184

Bet thal major

Absent beta chain

Extramedullary hematopoeisis (hepatosplenomegaly)

Skull deformations (medullary expansion)

186

Mechanism of eosinopenia with GC use

Sequesters eosinophils in lymph nodes

187

Decreased platelet count + increased bleeding time

Bernard-Soulier (no GpIb)

ITP (Ab against GpIIb/IIIa)

TTP (no ADAMTS13 = vWf MMP)

189

Decreased platelet count + prolonged BT and PT/PTT

DIC

190

Wrist and foot drop in kid

Lead poisoning

 

(give SUCCIMER)

191

Infectious cause of megaloblastic anemia?

Diphyllobothrium latum (fish tapeworm) = B12 deficiency

192

Leukemia most associated with DIC

Promyelocytic leukmia (AML M3)

 

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

194

Recurrent otitis media in kid (if cancer)

Langerhans cell histiocytosis

 

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

195

Burkitt lymphoma translocation

t(8:14)

196

Heparin, histamine, eosinophil chemotactic factor

Mast cells

198

CD28+

Co-stimulatory signal for T cell activation

199

Eosinophils contain:

Arylsulfatase, heparinase

200

Hemolytic anemia + venous thrombis + pancytopenia

PNH (no GPI anchor for DAF)

201

t(11:14)

Mantle cell lymphoma; cyclin D1 to Ig

202

How do nephrotic syndrome and renal failure cause hypercoagulability?

Loss of ATIII in urine = increased factors II and X

203

Diagnosis of PNH

CD55/59 - on flow cytometry

205

Treatment of hairy cell leukemia

Cladribine (2-CA)

 

Adenosine analog resistant to ADA

206

3 functions of BK

1. Vasodilation

2. Pain

3. Increase permeability

207

CD10+

ALL

 

(Pre-B cell marker)

208

Labs in hemochromatosis

Increased iron and ferritin and % transferrin sat

Decreased transferrin/TIBC

209

Point mutation in SCD

Glutamic acid --> valine at position 6 of beta gene

210

Two RBC pathologies in thalassemia?

Basophilic stippling

Target cells

211

Causes?

DIC

TTP/HUS

Prosthetic valve

212

Marker of macrophage

CD14 (binds LPS)

213

Skin necrosis after warfarin administration

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

215

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

Coporphobilinogen

216

Best prognosis for Hodgkin's?

Lymphocyte rich form

217

RBC pathology in abetalipoproteinemia

Acanthryocyte (spiny cell)

218

CD25+

Treg

219

Alk phos, collagenase, lactoferrin, lysozyme

PMN small, numerous granules

220

Triad of PNH

Hemolytic anemia + venous thrombus + pancytopenia

221

Defect in Glanzmann thromboasthenia

GpIIb/IIIa receptor

222

DIC vs. TTP

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

Pseudo-Pelger-Huet anomaly

Bilobed neutrophils (hyposegmented) typically following chemo

224

Two jobs of plasmin

1. Degrades fibrin

 

2. Activates complement (C3 --> C3a)

225

What causes bite cells?

Removal of precipitated Hb by phagocytes

226

Who gets adult T cell lymphoma?

IVDA (HTLV-1)

227

Urobilinogen vs. unconjugated bilirubin in urin

Urobilinogen = IV hemolysis

 

Unconjugated bilirubin = EV hemolysis (also jaundiced)

229

Mycosis fungoides marker

CD4+

230

1-2 alpha chain allele deletions

No clincally significant anemia

231

M spike = IgM

Waldenstrom's macroglobulinemia (hyperviscosity syndrome)

232

Treatment of AIP

Glucose and heme, which inhibit ALA synthase

233

t(14:18)

Diffuse large B-cell lymphoma

 

Follicular lymphoma, bcl-2

234

Unique to Tregs

FoxP3, CD25+

235

Hb barts

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