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Hematology Boards > Benign hematology > Flashcards

Benign hematology Flashcards

1
Q

Blood group that Parvo binds to

A

P

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2
Q

Salmonella infection in SS manifestations

A

Osteomyelitis of the long bones

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3
Q

Indications for HU in SSD

A

> = 3 VOC per year
Recurrent ACS
Stroke history

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4
Q

Goal HbS for exchange

A

<30%

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5
Q

Goal HbS for chronic exchange transfusion

A

<30%

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6
Q

Preoperative simple transfusion goal in SS

A

Hgb > 10

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7
Q

Cancer risk for SS trait

A

Renal medullary cancer

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8
Q

Goal HbF for HU in SSD

A

20%

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9
Q

Hemoglobin M pathophysiology

A

Mutation in heme pocket and form iron-phenolate resistant to reduction in divalent state

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10
Q

Hemoglobin M methemoglobinemia range

A

15-30%

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11
Q

Hemoglobin M prognosis

A

life expectancy unchanged

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12
Q

Hemoglobin M clinical manifestations

A

Cyanosis not responding to methylene blue

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13
Q

Oxygen dissociation curve shift in methemoglobin

A

To the left - non-methemoglobin in ferrous state and have increased affinity for oxygen

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14
Q

Clinical manifestations of methemoglobinemia

A

Cyanosis with normal pO2

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15
Q

Culprits for acquired methemoglobinemia (4)

A

Bactrim
dapsone
aniline dyes
rasburicase

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16
Q

Congenital deficiency causing methemoglobinemia

A

NADH-cytochrome b5 reductase

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17
Q

Congenital risk factors for methemoglobinemia (2)

A

G6PD deficiency

Pyruvate kinase deficiency

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18
Q

HbCC disease manifestations (3)

A

Mild hemolytic anemia
splenomegaly
prone to gallstones

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19
Q

HbSC disease manifestations (4)

A

Less VOC
More retinopathy
Priapism
Ischemic necrosis of bone

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20
Q

How to distinguish Hb C from Hb A2

A

citric agar for electrophoresis

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21
Q

Pyruvate kinase deficiency inheritance pattern

A

AR

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22
Q

Gene mutation for pyruvate kinase deficiency

A

PKLR

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23
Q

Deficiencies with pyruvate kinase deficiency

A

pyruvate and ATP

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24
Q

Symptoms of pyruvate kinase deficiency (4)

A

Splenomegaly
Gallstones
Jaundice
Leg ulcers

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25
Q

Sickle beta+ Hb electrophoresis

A

HbS: moderate
HbA: low
HbF: modeate
HbA2: moderate

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26
Q

Sickle beta0 Hb electrophoresis

A

HbS: high
HbA: absent
HbF: moderate

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27
Q

HbSS Hb electrophoresis

A

HbS: very high
HbA: absent
HbA2: very low
HbF: moderate

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28
Q

Sickle-HbC electrophoresis

A

HbS: moderate
HbC: moderate
HbS: moderate
HbF: mild

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29
Q

Direct Coombs in drug induced AIHA

A

IgG-. C3+

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30
Q

Phosphoglycerate kinase deficiency gene

A

PGK1

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31
Q

Phosphoglycerate kinase deficiency clinical manifestations

A

non-spherocytic hemolytic anemia

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32
Q

Spleen change after RBC transfusion in splenic sequestration crisis

A

Spleen shrinks

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33
Q

Treatment of splenic sequestration crisis in SSD

A

Careful transfusion

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34
Q

Treatment of pulmonary HTN in SSD

A

HU

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35
Q

Complications of zinc over supplement

A

Copper deficiency and pancytopenua

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36
Q

ESA resistannce in ESRD patient cause

A

Secondary hyperparathyroidism

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37
Q

Manifestations of hereditary elliptocytosis

A

hemolysis

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38
Q

Most common Coombs for warm agglutinin AIHA

A

IgG+, C3+

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39
Q

Target of warm agglunin antibody

A

Rh epitope

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40
Q

MCV and MCHC in cold agglutination

A

Both elevated

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41
Q

Red cell antigen for cold agglutination disease

A

I

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42
Q

Where red cell destruction takes place in cold agglutination disease

A

Liver

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43
Q

Treatment of cold agglutinin disease

A

Treat underlying disease

Steroids and splenectomy not effective

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44
Q

Treatment of refractory idiopathic cold agglutinin disease

A

Rituximab

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45
Q

Urgent treatment of cold agglutinin disease

A

Plasmapharesis

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46
Q

Paroxysmal cold hemoglobinuria test

A

Donath-Landsteiner Ab

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47
Q

Mechanism of antibiotic associated AIHA

A

Hapten reaction

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48
Q

Mutations for hereditary spherocytosis (3)

A

Spectrin
Ankyrin
Band 3

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49
Q

CBC finding for hereditary spherocytosis

A

Elevated MCHC

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50
Q

Confirmatory test for hereditary spherocytosis

A

Eosin-5-maleimide (EMA) is low

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51
Q

Treatment of hereditary spherocytosis in Jehovah’s witness

A

Splenectomy

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52
Q

Inheritance for hereditary elliptocytosis

A

AD

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53
Q

Peripheral blood findings for lack of Kell Ab

A

Acanthrocytes

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54
Q

Coombs negative hemolytic anemia, absence of morphologic abnormalities cause

A

Pyruvate kinase deficiency

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55
Q

Inheritance of PK deficiency

A

X linked

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56
Q

Peripheral blood smear in G6PD deficiency

A

Heinz bodies and bite cells

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57
Q

Enzyme inhibited by lead poisoning

A

Pyramidine-5-nucleotidase

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58
Q

Drugs associated with interferon

A

Gemcitabine, bevacizumab
Tacriolimus, cyclosporine
Cocaine

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59
Q

Metformin vitamin deficiency

A

B12

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60
Q

Major complication of thalassemia intermedia

A

osteoporosis

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61
Q

High/normal B12 but deficiency syndrome in children

A

Transcobalamin II deficiency

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62
Q

Hereditary pyropoikilocytosis symptoms

A

Severe hemolytic anemia

Young age of onset

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63
Q

When to suspect methemoglobinemia

A

Cyanosis with normal pO2

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64
Q

Treatment of cold agglutinin secondary to mycoplasma

A

Azithromycin

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65
Q

Majeed syndrome clinical characteristics (3)

A

Recurrent osteomyelitis
Sweet syndrome
Dyserythropoietic anemia

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66
Q

Majeed syndrome mutation

A

LPIN2

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67
Q

Target of Donath-Landsteiner Ab in PCH

A

P antigen

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68
Q

Oral iron with less GI side effects

A

Ferric maltol

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69
Q

HbE/beta thalassemia clinical characteristics

A

South Asia

Mild anemia

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70
Q

Cause of INH induced anemia

A

δ-Aminolevulinate-synthase inhibition

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71
Q

HbS/hereditary persistence of fetal Hb symptoms

A

Asymptomatic

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72
Q

HbH clinical manifestations

A

Mild anemia

Iron overload

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73
Q

Hb Barts condition

A

Hydrop fetalis or HbH

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74
Q

Hb Barts oxygen affinity

A

Extremely high

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75
Q

HbH disease alpha globin genes

A

(–,–), (–,a)

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76
Q

Hb Constant Spring defect

A

Point mutation leading to elongation of alpha globin chain

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77
Q

Hb Constant Spring clinical manifestations

A

Severe anemia

Normocytic

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78
Q

Thal minor designation

A

Hb b/b+ or Hb b0/b

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79
Q

Thal intermediate designation

A

Hb b+/b+ or b0/b+

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80
Q

Deficient enzyme in congenital methemoglobinemia

A

Cytochrome b5 reductase

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81
Q

Gene responsible for alpha thal

A

ATRX

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82
Q

Abetalipoproteinemai RBC morphology

A

acanthrocytes

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83
Q

Urgent treatment for babesiosis

A

Exchange transfusion plus atovaquone+azithro

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84
Q

Clinical characteristics for HbE beta-0

A

Similar to beta-thal intermediate

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85
Q

RDW in alpha and beta thal

A

normal

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86
Q

Treatment of congential ringed sideroblasts

A

Vit B6 (pyridoxine)

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87
Q

Hereditary pyrimidine 5’-nucleotidase deficiency treatment

A

Splenectomy

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88
Q

Origin of red cell destruction in colg agglutinin disease

A

Liver

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89
Q

Hemoglobin H cause

A

loss of 3 alpha chains

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90
Q

Hb Constant Spring

A

HbH with 2 deletions and 1 mutation

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91
Q

Hb Constant Spring clinical manifestations

A

Severe anemia

Splenomegaly

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92
Q

Moderate hemopphilia levels

A

1-5%

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93
Q

Dose of FVIII for prophylaxis or treatment

A

50U/kg

94
Q

Treatment of mild hemophilia with mild bleeding

A

aminocaproic acid

95
Q

Level maintained for hemophilia and major surgery

A

Prior to surgery: 100%

After surgery: 50%

96
Q

Level maintained for hemophilia prophylaxis

A

> 1%

97
Q

Number of Bethesda units for factor to not work

A

5

98
Q

Treatment of Hem A with inhibitor (3)

A

Recombinant VIIIa
Porcine VIII
Prothrombin concentrate (FEIBA)

99
Q

Prolonged PT and PTT in amyloidosis cause

A

Factor X inhibitor

100
Q

Findings of aVWD

A

Normal Ag
Decreased ristocetin
Decreased multimers

101
Q

Level for VW for epidural

A

50 IU/dL

102
Q

Blood type with low vW

A

O

103
Q

Treatment of bleeding in vWD Type 2B

A

vWF concentrate + platelets

104
Q

Treatment of Factor X def bleeding

A

Factor X concentrate OR

PCC

105
Q

Type I VWD inheritance

A

AD

106
Q

Type I VWD vs Type III VWD

A

Some VWF vs none

107
Q

Heye’s syndrome bleeding diathesis

A

Acquired Type IIA VWD

108
Q

Type IIA VWD findings

A

Absent large multimers

109
Q

Goal FVIII level for joint bleeding

A

peripheral - 50%

proximal - 100%

110
Q

VWD Type IC mechanism

A

Increased clearance of VWF

111
Q

Treatment of Type IC VWD

A

Humate (FVIII + VWF)

112
Q

Treatment of Type I VWD during pregnancy

A

Monitor levels (will rise during pregnancy)
VWF > 50
DDAVP soft contraindication because of uterine contractions
Continue replacement for 3 weeks

113
Q

Treatment of hemophilia A bleed on emicizumab

A

FVIII

114
Q

Glanzmann’s thrombocythemia defect

A

GPIIb/IIIa

115
Q

INR to give Vit K in nonbleeding warfarin

A

10

116
Q

Factor deficiency leading to elevated PT and PTT but normal thrombin time (2)

A

Factor V

Factor X

117
Q

Use of reptilase time

A

Not sensitive to anticoagulants

118
Q

Duration of anticoagulation for high risk superficial vein thrombosis

A

45 days

119
Q

Clinical manifestations Factor V

A

Minor bleeding

Elevations in PT and PTT

120
Q

Treatment of bleeding with Factor V deficiency and goal FV level

A

FFP

20%

121
Q

5M urea clot lysis assay positive disease

A

Factor XIII deficiency

122
Q

HELLP vs severe pre-eclampsia timing

A

Third trimester (HELLP) second for severe preeclampsia

123
Q

Prolonged thrombin time or reptilase time meaning

A

low and dysfunctional fibrinogen

124
Q

VW Type IIB diagnosis

A

Binds platelets - increased risocetin activity

125
Q

Treatment of bleeding in VW Type IIB

A

Humate P (VW replacement)

126
Q

VW Type IIN diagnosis

A

Nothing to do with FVIII (severe reductions because of degrading of FVIII)

127
Q

Inheritance for Type I VW

A

AD

128
Q

Inheritance for Type III VW

A

AR

129
Q

Use of DDAVP in VW - which types

A

Type I and IIA with mild procedures

130
Q

Treatment of FII deficiency associated with LAC

A

Observation, can resolve

131
Q

Location of May-Thurner related clot

A

Left iliac

132
Q

Type IIM VW diagnosis

A

Multimers normal present but decreased

133
Q

Strong APLS Ab for thrombosis

A

LA

134
Q

Least thrombogenic contraceptive

A

IUD

135
Q

Inheritance of antithrombin deficiency

A

AD

136
Q

Deficiency associated with purpura fulminans

A

Protein C deficiency

137
Q

Indications for extended post-surgical prophylaxis (4 weeks)

A

abdominal or pelvic surgery for cancer

138
Q

management of pregnant homozygous FVL without family or personal VTE history

A

observation + antepartum enoxaparin

139
Q

Treatment of APLS with prior fetal loss during subsequent pregnancy

A

enoxaparin + ASA

140
Q

Brain mets that can bleed (4)

A

Thyroid
RCC
Chorio
Melanoma

141
Q

Betrixaban indication

A

Hospital VTE prophylaxis

142
Q

Duration of AC for pregnancy induced VTE

A

6 weeks postpartum and for 3 months total duration

143
Q

Indications for iron chelation in beta thal (3)

A

Ferritin > 1000
liver iron concentration > 3mg/g
20-25 transfusion

144
Q

Juvenile hemochromatosis gene

A

HJV

145
Q

H63D homozygous HH manifestations

A

biochemical but not clinical HH

146
Q

Goal ferritin in HH

A

<100

147
Q

Goal ferritin in chelation for iron overload

A

<1000

148
Q

Test for porphyria

A

urine porphobilogen (PBG)

149
Q

Target of Hemin

A

ALAS1 synthesis

150
Q

Treatment to prevent neonatal hemochromatosis in pregnant woman

A

IVIG

151
Q

Iron overload with normal transferrin sat and neurological symptom diagnosis

A

Aceruloplasminemia

152
Q

Iron refractory iron deficiency anemia gene

A

TMPRSS6

153
Q

Iron deposition in Kuppfer cells disease

A

Classical ferroportin disease

154
Q

Iron deposition in Kuppfer cells disease gene

A

FPN

155
Q

Only HH gene that is AD

A

FPN1

156
Q

Management of porphyria cutenea tarda

A

phlebotomy

157
Q

Only porphyria inherited as AR

A

δ-ALA dehydratase porphyria

158
Q

Treatment of porphyria cutaneous tarda refractory to phlebotomy

A

Hydroxychloroquine

159
Q

Treatment of acute hepatic porphyria

A

Givosiran

160
Q

Defect in Glazmann’s thrombocythemia

A

integrin alpha2beta3

161
Q

Defect in Bernard Soulier

A

GP Ib/IIa

162
Q

Platelets in Bernard Soulier

A

Thrombocytopenia, large platelets

163
Q

Findings on platelet aggregation studies for storage disease

A

Decreased aggregation in response to ADP, collagen, epinephrine

164
Q

Hermansky Pudlack symptoms (3)

A

1) oculocutaneous albinism
2) bleeding diathesis
3) pulmonary fibrorsis

165
Q

GP Ib/IX/V function

A

binds platelet to endothelium

166
Q

Upshaw–Schulman deficiency

A

ADAMTS13

167
Q

Clopidogrel hematology disease association

A

TTP

168
Q

Quebec Platelet disorder deficiency

A

Urokinase plasminogen activator

169
Q

Treatment of bleeding in Glanzmann’s disorder 1st and 2nd line

A

1) platelets

2) Recombinant FVIIa

170
Q

Target of antibody in post-transfusion purpura

A

HPA-1a

171
Q

MYH9-related disorders manifestations (3)

A

Thrombocytopenia
Dohle-like bodies
Sensorineal hearing loss

172
Q

Glanzmann’s thrombocythemia platelet aggregation study findinbg

A

Aggregate to risocetin, but not to ADP, collagen, epinephrine

173
Q

Treatment of fetal and neonatal alloimmune thrombocytopenia

A

IVIG in mother at 20 weeks

174
Q

Factors in cryopreciptate (4)

A

fibrinogen
FVIII
FXIII
vWF

175
Q

Most common virus acquired from transfusion

A

HBV

176
Q

Management of AHTR (2)

A

Hydration

Alkaline urine

177
Q

Treatment of neonate with alloimmune thrombocytopenia

A

Washed platelets from mother

178
Q

Most common blood group antigens outside of ABO and Rh

A

Kell

179
Q

Major mismatch situation

A

Recipient has antibodies to donor

180
Q

Minor mismath situation

A

Donor has antibodies to recipient

181
Q

Bidirectional situation

A

Both recipient antibodies to donor and donor antibodies to recipient

182
Q

Treatment of cyclic neutrophilia in children

A

GCSF

183
Q

Severe congenital neutropenia gene

A

ELANE

184
Q

Chediak-Higashi morphology

A

Neutrophils with large lysosome inclusions

185
Q

Test for CGD (2)

A

NBT or DHR

186
Q

Treatment of CGD

A

Interferon gamma-1b

187
Q

Physical findings in Fanconi anemia (3)

A

Microcephaly
Skin hyperpigmentation
Short stature

188
Q

First line treatment for AA (non transplant)

A

Horse ATG, cyclosporine, +/- eltrombopag

189
Q

Treatment of chronic parvovirus causing pure red cell aplasia

A

IVIG

190
Q

Barth syndrome symptoms (3)

A

neutropenia
dilated cardiomyopathy
skeletal myopathy

191
Q

WHIM syndrome gene defect

A

CXCR4

192
Q

WHIM syndrome treatment

A

Plerixifor

193
Q

When to treat PNH clone in AA

A

> 50%

194
Q

Gene associated with dyskeratosis congenita (2)

A

TERT

TERC

195
Q

Gene defect in Diamond-Blackfan

A

RPS19

196
Q

Pancreatic exocrine malfunction with neutropenia disease

A

Shwachman-Diamond syndrome

197
Q

RDW in AOCD

A

Normal

198
Q

Most common three alloantibodies to RBC

A

Kell, C, and E

199
Q

Who to give postsplenectomy ppx to

A

age <16 or >50

200
Q

AC for pregnant woman with provoked VTE and any thrombophilia

A

Antepartum and postpartum AC

201
Q

AC for pregnant woman with APLS

A

Lovenox 40 and ASA 81

202
Q

Treatment of post-transfusion purpura

A

IVIG

203
Q

AC for pregnant woman with heterozygous FVL

A

Observation

204
Q

% febrile neutropenia where you give GCSF

A

20%

205
Q

Mechanism of iron overload in ineffective erythropoiesis

A

Increased erythroferrone production by erythroblasts

206
Q

Deferasirox monitoring (3)

A

CMP (Cr and LFTs)
Opthalmologic exam (annual)
Auditory (annual)

207
Q

Most common genotype for clinical HH

A

C282Y homozygous

208
Q

Treatment of SS priapism

A

Urology evaluation

209
Q

Back pain in Thal Major patient cause

A

EMH in spine

210
Q

PK levels after splenectomy

A

Can be falsely low

211
Q

Causes of secondary porphyria cutaneous tarda (2)

A

HIV

Alcohol

212
Q

Indications for treatment of PNH

A

more than mild symptoms

213
Q

Duration of AC ppx after cancer surgery

A

28 days

214
Q

Postpartum cerebral sinus vein thrombosis associated with moderate hemorrhage

A

AC

215
Q

When to use procine rFVII

A

inhibitor level <20

216
Q

Treatment of FVII deficiency

A

rFVIIa

217
Q

Prolonged TT with prolonged PT and PTT

A

fibrinogen defect

218
Q

Differentiating Glanzmans thrombothemia and Bernard Soulier

A

Bernard Soulier has thrombocytopenia

219
Q

Platelet count in Glanzmans thrombothemia

A

Normal

220
Q

Intraop AC in patient with hx of HITT but no PF4 Ab

A

Heparin

221
Q

Diamond-Blackfan manifestation

A

Pancytopenia, family history of leukemia

222
Q

Test for Diamond-Blackfan

A

erythrocyte adenosine deaminase concentration

223
Q

Test for Fanconi anemia

A

Chromosome breakage analysis

224
Q

Erdheim-Chester disease mutation

A

BRAF V600E

225
Q

Type 2A VW findings

A

Absent large multimers

226
Q

Target VW level for neuroaxial anesthesia

A

0.5-1.5

227
Q

VW Activity:Ag ratio for Type 2

A

<0.7

228
Q

Diagnosis of VWD levels

A

activity of antigen <30% or <50% with bleeding

229
Q

VW Type that you cannot use desmopression

A

IIB

230
Q

Response to risocetin Bernard Soulier vs Glanzmanns

A

Glansmanns aggregates

Bernard does not

Hematology Boards (2 decks)

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