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PSBIM > HEMATOLOGY > Flashcards

HEMATOLOGY Flashcards

1
Q

Internal causes of posthemorrhagic anemia (4)

A
  • GI bleeding
  • Rupture of spleen
  • Rupture of an ectopic pregnancy
  • Subarachnoid hemorrhage
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2
Q

Dominant feature of the 1st clinical/pathophysiological stage after blood loss

A

Hypovolemia

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3
Q

Major threats of the 1st clinical/pathophysiological stage after blood loss (2)

A
  • Loss of consciousness

* Acute renal failure

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4
Q

Dominant feature of the 2nd clinical/pathophysiological stage after blood loss

A

Anemia (due to hemodilution)

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5
Q

If 3 days after acute blood loss, hemoglobin is at 7 g/dL, how many percent of the blood has been lost?

A

About 50%

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6
Q

Dominant feature of the 3rd clinical/pathophysiological stage after blood loss

A

Elevated reticulocyte count and EPO levels

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7
Q

The most frequent neoplastic disease in children

A

ALL

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8
Q

Peak age of ALL

A

3–4 years of age

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9
Q

Congenital chromosomal abnormality that have a twentyfold increased incidence of leukemia

A

Down syndrome

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10
Q

The etiological agent for adult T-cell leukemia/ lymphoma

A

Human T-cell leukemia virus I (HTLV-I)

An aggressive adult T-cell leukemia

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11
Q

Etiologic agent of an endemic African type of Burkitt’s lymphoma

A

Epstein-Barr virus

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12
Q

The major criteria to subdivide ALL into B-cell lineage or T-cell lineage (T-ALL) leukemias

A

Immunological markers

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13
Q

Major aim of classification of acute leukemia

A

to distinguish between AML and ALL

Different treatment approaches and drug sensitivities

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14
Q

to distinguish between AML and ALL

Different treatment approaches and drug sensitivities

A

Acute leukemia (ALL)

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15
Q

Biopsy of the bone marrow demonstrate marked hypercellularity with replacement of fat spaces and normal elements by infiltration with leukemic cells

A

ALL

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16
Q

An essential routine diagnostic measure for ALL to rule out CNS leukemia

A

Lumbar puncture

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17
Q

Lumbar puncture is restricted to leukemic patients with (3)

A
  1. Adequate platelet count (>20 × 109/L)
  2. Absence of manifest clinical hemorrhage
  3. Without a high white blood cell count
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18
Q

This should be given to all at the first lumbar puncture in leukemic patients

A

Intrathecal methotrexate

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19
Q

French-American-British classification morphology of ALL that has clinical and prognostic relevance

A

L3 morphology

L1 and L2 - no clinical consequence

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20
Q

French-American-British classification morphology of ALL that is indicative of mature B-ALL (aka Burkitt’s leukemia)

A

L3 morphology

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21
Q

Positive marker in leukemia is considered if ____ of the cells are stained with the monoclonal antibody

A

> 20%

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22
Q

Which is more common? B cell ALL or T cell ALL?

A

B cell

More than 70% of adult ALLs

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23
Q

The most frequent immunological subtype of ALL

A

Common ALL

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24
Q

ALL antigen

A

gp100/CD10 – a glycoprotein

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25
Q

ALL subtype that is characterized by the expression of cytoplasmic immunoglobulin

A

Pre-B-ALL (early B-ALL)

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26
Q

Also termed early B-precursor ALL

A

Pro-B-ALL

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27
Q

A leukemia that was formerly termed non-T, non-B-ALL, or null-ALL

A

Pro-B-ALL

Neither T-cell nor B-cell features could be demonstrated

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28
Q

Markers of Pro-B-ALL (2)

A
  1. Tdt (terminal) deoxynucleotidyl transferase

2. CD19

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29
Q

All T Cell lineage ALL has these antigens (2)

A
  1. T-cell antigen (gp40, CD7)

2. Cytoplasmatic or surface CD3

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30
Q

Leukemia wherein markers of lymphoid and myeloid lineages are co-expressed on the same leukemic cells

A

Biphenotypic or Mixed Leukemias

Without the typical phenotype of either ALL or AML

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31
Q

Newly defined ALL entities with poor prognosis (2)

A
  1. Ph-like ALL

2. Early T-precursor ALL

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32
Q

Dasatinib is a

A

BCR-ABL inhibitors

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33
Q

Ruxolitinib is a

A

JAK2 inhibitors

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34
Q

Detection of residual leukemic cells, not recognizable by light microscopy

A

Minimal Residual Disease

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35
Q

Methods for determining MRD (3)

A
  1. Flow cytometry
  2. RT-PCR
  3. Detection of fusion genes associated with chromosomal abnormalities
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36
Q

T or F. Molecular response can be evaluated only for patients in complete cytological remission from ALL

A

True

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37
Q

The most relevant independent prognostic factor for disease-free survival and overall survival from ALL

A

Molecular complete response/molecular remission

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38
Q

Prognostic parameters of ALL (4)

A
  1. Age
  2. White blood cell count
  3. Specific immunophenotypes
  4. Cytogenetic and genetic aberrations
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39
Q

Standard-risk patients of ALL is defined as _____.

A

without any poor-risk factors

With a good chance of cure by chemotherapy

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40
Q

High-risk patients of ALL is defined as _____.

A

with one or more of poor risk factors

Most often candidates for a stem cell transplant in first complete remission

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41
Q

Outcome of ALL is strictly related to (2)

A
  1. Age of the patient

2. Treatment protocols

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42
Q

Maintenance therapy for ALL (3) and duration

A
  1. 6-Mercaptopurine
  2. Methotrexate
  3. TKI (Ph-positive ALL)

Duration: 2 – 2.5 years

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43
Q

Without some form of prophylactic CNS-directed therapy, around ___ of adults with ALL developed CNS leukemia

A

30%

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44
Q

Tyrosine kinase inhibitors that cross the BBB (2)

A

Dasatinib and ponatinib

Imatinib and nilotinib – do not cross the BBB

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45
Q

The most promising option to achieve a remission of CNS leukemia

A

Allogeneic stem cell transplantation

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46
Q

T or F. Sibling donors is much more preferred than unrelated donors in stem cell transplantation

A

False. There is a shift from sibling donors to matched unrelated donors or haploidentical transplants from relatives

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47
Q

Indications for stem cell transplantation in first remission:

A
  1. Persistent MRD

2. All high-risk patients either defined by conventional clinical prognostic factors or by MRD positivity

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48
Q

Autologous stem cell transplantation in first remission of ALL is restricted to these patients (3)

A
  1. Ph+ ALL
  2. MRD negative
  3. Older patients
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49
Q

The only curative option for all relapsed adult ALL patients

A

Allogeneic stem cell transplant

Recommended to all patients in second or later complete remission

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50
Q

1st generation TKI

A

Imatinib

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51
Q

2nd generation TKI

A

Dasatinib, nilotinib

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52
Q

3rd generation TKI

A

Ponatinib

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53
Q

Anti CD20 used in immunotherapy of ALL

A

Rituximab

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54
Q

Anti CD22 used in immunotherapy of ALL (2)

A
  1. Inotuzumab ozogamicin

2. Epratuzumab

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55
Q

Anti CD19 and CD3 used in immunotherapy of ALL

A

Blinatumomab

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56
Q

Leukemic cells not detectable by light microscopy (<5% blast cells in bone marrow)

A

Complete (hematologic) remission

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57
Q

Patient in complete remission, MRD not detectable, ≤0.01% = ≤1 leukemia blast cell in 10,000

A

Complete molecular remission MRD-negativity

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58
Q

Patient in complete hematologic remission but not in molecular complete remission >0.01%

A

Molecular failure/ MRD-positivity

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59
Q

Patient still in complete remission had prior molecular complete remission. Leukemic blast cells detectable in bone marrow not detectable (<5%)

A

Molecular relapse/ MRD-positivity

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60
Q

> 5% ALL cells in bone marrow/blood after chemotherapy

A

Hematologic relapse

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61
Q

Targeted therapy for Ph/BCR-ABL+ ALL

A

TKIs

Imatinib, Dasatinib, Nilotinib, Bosutinib, Ponatinib

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62
Q

Targeted therapy for Ph-/BCR-ABL-like ALL

A

Dasatinib and Ruxolitinib

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63
Q

Most common form of anemia

A

hypoproliferative anemia

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64
Q

reticulocyte index in hypoproliferative anemia

A

<2-2.5

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65
Q

Most common form of hypoproliferative anemia

A

iron deficiency anemia

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66
Q

Normochromic, normocytic anemias (6)

A
  1. early stage of IDA
  2. anemia of acute and chronic inflammation
  3. renal disease
  4. protein malnutrition
  5. endocrine deficiencies
  6. marrow damage
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67
Q

Transferrin that has the highest affinity for transferrin receptors

A

diferric transferrin

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68
Q

Storage protein to which excess iron binds

A

apoferritin

forms ferritin

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69
Q

average life span of RBC and the turn over rate per day

A

120 days

0.8-1.0%

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70
Q

Amount of iron needed to replace the RBCs lost through senescence

A

20 mg/day

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71
Q

Amount of dietary iron requirement per day (for men and women)

A

men - 10% of body iron content/year = 1 mg elemental iron daily

female of childbearing age - ~15% of body iron content/year = 1.4 mg elemental iron daily

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72
Q

During the last 2 trimesters of pregnancy, the daily iron requirements increase to

A

5-6 mg

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73
Q

Primary site of iron absorption

A

proximal small intestine

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74
Q

principal iron regulatory hormone that regulates the ferroportin transporter

A

hepcidin

low in IDA in order to facilitate increase absorption

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75
Q

Stages of iron deficiency (3)

A
  1. negative iron balance
  2. iron-deficient erythropoiesis
  3. IDA
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76
Q 
Laboratory finding in the first stage of iron deficiency in terms of:
serum ferritin
stainable iron on BMA
serum iron
TIBC
red cell protorphyrin
RBC morphology and indices
A

low :
serum ferritin
stainable iron on BMA

normal:
serum iron
TIBC
red cell protorphyrin

normochromic normocytic

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77
Q 
Laboratory finding in the 2nd stage of iron deficiency in terms of:
serum ferritin
stainable iron on BMA
serum iron
TIBC
red cell protorphyrin
TSAT
RBC morphology and indices
A 
serum ferritin - decreased 
stainable iron on BMA - decreased 
serum iron - decreased
TIBC - increased
red cell protorphyrin - increased
TSAT -decrease to 15-20%
RBC morphology and indices - microcytic, hypochromic
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78
Q

Laboratory finding in IDA in terms of: TSAT

A

Decreased TSAT to 10-15%

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79
Q

Normal values for:

Marrow iron stores
serum ferritin
TIBC
Serum iron
TSAT
marrow sideroblasts
RBC protoporphyrin
A 
Marrow iron stores: 1-3+
serum ferritin: 50-200 ug/L 
TIBC: 300-360 ug/dL
Serum iron: 50-150 ug/dL
TSAT: 30-50%
marrow sideroblasts: 40-60%
RBC protoporphyrin: 30-50 ug/dL
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80
Q

T or F. Erythropoietin therapy may cause iron deficiency

A

True. increase demand for iron

81
Q

IDA in adult male means

A

GI loss unti proven otherwise

82
Q

Signs of advanced tissue Fe deficiency (2)

A

koilonychia

cheilosis

83
Q

fissures at corner of the mouth seen in IDA

A

cheilosis

84
Q

spooning of fingernails seen in IDA

A

koilonychia

85
Q

represents amount of circulating iron bound on transferrin

A

serum iron

86
Q

indirect measure of the circulating transferrin

A

TIBC

87
Q

Transferrin saturation or TSAT is calculated by

A

serum iron x 100/TIBC

88
Q

ferritin accumulating in the RE cells and is less readily available source of iron

A

hemosiderin

89
Q

the most convenient laboratory test to estimate iron stores

A

serum ferritin levels

better indicator of iron overload than marrow iron stain

90
Q

serum ferritin level that is virtually diagnostic of absent body iron store

A

< 15 ug/L

91
Q

consists of 20-40% of developing erythroblast with visible cytoplasmic ferritin granules representing iron in excess of that needed for hemoglobin synthesis

A

sideroblasts

92
Q

Iron accumulates in dysfunctional mitochondria

A

ringed sideroblast - appear as a necklace ring around the nucleus

93
Q

ringed sideroblast is commonly seen in what condition

A

myelodysplastic syndromes

94
Q

Intermediate in the pathway of heme synthesis which accumulates within red cells when heme synthesis is impaired

A

protoporphyrin

95
Q

most common causes of increased levels of red cell protoporphyrin (2)

A
  1. absolute or relative iron deficiency

2. lead poisoning

96
Q

Causes of hypochromic, microcytic anemias (4)

A
  1. IDA
  2. thalassemias
    3 anemia of inflammation
  3. myelodysplastic syndromes (least common)
97
Q

Goals of therapy in IDA (2)

A
  1. anemia correction
  2. provide stores of 0.5 to 1 g of iron

treatment for period of 6-12 months after correction of anemia

98
Q

Amount of iron needed by an individual patient is calculated by the following formula:

A

BW (kg) x 2.3 x (15-Hgb (g/dL)) + 500 or 1000 mg

99
Q

Most distinguishing feature between true IDA and iron-restricted erythropoiesis associated with inflammation

A

serum ferritin

inflammation: 3-fold increase in serum ferritin

100
Q

acute infection can cause a fall in the Hgb levels of ____ within ____

A

2 to 3 g/dL

1-2 days

101
Q

Mechanism of anemia of acute infection

A

faster destruction of nearly senescent RBCs (hemolysis of RBCs near end of their life span)

102
Q

T or F. CKD sec to hypertensive kidney disease have more severe EPO deficiency for a given level of renal failure

A

false. DM

103
Q

Hormones that augment erythropoiesis

A

testosterone and anabolic steroids

castration and estrogen administration in males will decrease erythropoiesis

104
Q

If with decrease in Hgb while on EPO, consider the following pathology (4)

A
  1. infection
  2. iron depletion
  3. aluminum toxicity
  4. hyperparathyroidism
105
Q

a monoclonal proliferation of mature B lymphocytes defined by an absolute number of malignant cells in the blood (5 × 10^9/mL)

A

Chronic lymphocytic leukemia (CLL)

106
Q

Median age of diagnosis of CLL

A

71

primarily a disease of older adults

107
Q

T or F. CLL is linked to to radiation exposure

A

False. CLL is one of the only types of leukemia not linked to radiation exposure

108
Q

T or F. CLL is one of the most familial-associated malignancies.

A

True. first-degree relative of a CLL patient has an 8.5-fold elevated risk of developing CLL than the general population

109
Q

Distinction of CLL from normal B cells

A

BCR signaling

110
Q

BCR signaling of CLL that is different from normal B cells

A
  1. low-level IgM expression
  2. variable response to antigen stimulation
  3. tonic activation of antiapoptotic signaling pathways that promote tumor survival
111
Q

One of the most influential prognostic factors identified in this disease is the mutational status of the immunoglobulin heavy chain variable (IGHV) region

A

CLL

112
Q

Because IGHV sequencing was initially cumbersome to perform, a number of surrogate factors have been identified; however, none yet have been shown to be equal or superior to IGVH sequencing. The most prevalent of surrogate markers for CLL are (3)

A

Zap-70 expression
ZAP-70 methylation
surface CD38 expression

113
Q

Besides IGHV mutational status, _______ are the most robust prognostic factor clinically available in CLL

A

recurrent cytogenetic abnormalities

The most well-characterized abnormalities include del(13) (q14.3), trisomy 12, del(11)(q22.3), and del(17)(p13.1)

114
Q

Cytogenetic abnormalities in CLL that is associated with more indolent disease, prolonged survival, and good response to traditional therapies

A

del(13)(q14.3)

115
Q

Cytogenetic abnormalities in CLL that results in deletion of the ATM gene and is associated with bulky lymphadenopathy and aggressive disease in young patients, with inferior prognosis, more rapid progression to symptomatic disease, and shorter survival

A

del(11) (q23.3)

116
Q

Cytogenetic abnormalities in CLL that results in loss of one allele of the tumor suppressor TP53 and is associated with the poorest prognosis in CLL with rapid disease progression, poor response to traditional therapies, and shorter survival

A

del(17)(p13.1)

117
Q

Mutation in what gene in CLL that is commonly associated with trisomy 12

A

NOTCH1

118
Q

Mutations have been associated with lower sensitivity of CLL to CD20 antibody therapy and increased risk of transformation to aggressive diffuse large B-cell lymphoma (DLBCL; Richter’s transformation).

A

NOTCH1

119
Q

The most common cytogenetic abnormality in CLL

A

del(13)(q14.3)

120
Q

When there is elevated total white blood cell (WBC) count with lymphocytic predominance or a normal WBC with a differential showing a lymphocytosis and a blood disorder is suspected, what is the next step?

A

Flow cytometry

121
Q

In cases in which the clonal B cell count based on flow cytometry is ≥5 × 10^9/L, the diagnosis is

A

CLL

no further workup is needed to confirm the diagnosis of CLL

122
Q

A semantic designation from CLL that denotes a primarily tissue-based disease rather than bone marrow/blood-based disease

A

small lymphocytic lymphoma (SLL)

small clonal proliferation of CLL cells in the peripheral blood but will also have lymphadenopathy or splenomegaly

123
Q

Patients who do not meet the diagnostic criteria for CLL based on quantification of clonal B cells in the peripheral blood and who do not have associated signs of CLL including lymphadenopathy, organomegaly, or cytopenias have a disorder known as

A

monoclonal B-cell lymphocytosis (MBL)

which is now thought to precede every case of CLL but not all MBL progresses to CLL

124
Q

leading cause of both disease-related morbidity and death in patients with CLL

A

Infections

125
Q

The most common types of cancers seen in CLL are (3)

A

skin cancers, prostate, and breast cancers

All CLL patients should be counseled on the use of sunscreen while outdoors and should undergo preventative skin examinations

126
Q

Most common autoimmune complications in CLL

A

Autoimmune cytopenias most commonly AIHA

Second most common is immune thrombocytopenia

127
Q

Sydroe of combination of AIHA and ITP

A

Evan’s syndrome

128
Q

In CLL, treatment for ITP is usually instituted when (3)

A
  1. platelet levels drop to 20–30,000
  2. Evidence of bleeding complications
  3. need for invasive procedures
129
Q

One of the most devastating complications of CLL wherein there is transformation of CLL to an aggressive lymphoma

A

Richter’s transformation

130
Q

CLL most commonly transform into what lymphoma

A

DLBCL

131
Q

Risk factors for Richter’s transformation (4)

A
  1. bulky lymphadenopathy
  2. NOTCH1 mutations
  3. del(17)(p13.1)
  4. specific stereotyped IGVH usage
132
Q

1st diagnostic test in suspected cases of Richter’s transformation

A

18FDG-PET/CT (fluorodeoxyglucose–positron emission tomography combined with computed tomography) scan

Standardized uptake values (SUV) <5 is consistent with CLL and can rule out Richter’s trans- formation in many cases. SUV >5 are suspicious for Richter’s transformation, with SUV ≥10 very concerning

133
Q

two widely used staging systems in CLL

A

Rai staging system
Binet system

Both characterize CLL on the basis of disease bulk and marrow failure

134
Q

T or F. Imaging and bone marrow analysis is a requirement in staging CLL

A

False. Staging only rely on physical examination and laboratory studies

135
Q

standard therapies for CLL

A

Chemotherapy and chemoimmunotherapy

136
Q

For CLL patients who are young (≤65 years), the gold standard for therapy is

A

Fludarabine + cyclophosphamide + rituximab (FCR)

nucleoside analogue fludarabine, the alkylator cyclophosphamide, and the anti-CD20 monoclonal antibody rituximab (FCR)

137
Q

For older CLL patients or those with multiple comorbidities, standard for therapy is

A

Chlorambucil + obinutizimab OR bendamustine + rituximab

138
Q

first agent to show a survival advantage in CLL

A

Rituximab

139
Q

a reversible, p110 delta isoform-specific PI3K inhibitor used in treatment of CLL

A

Idelalisib

B-Cell Receptor Signaling Inhibitors

140
Q

a relatively selective, irreversible inhibitor of Bruton’s tyrosine kinase (BTK) in CLL

A

Ibrutinib

This target is attractive because, unlike other kinases in the BCR pathway, BTK does not have natural redundancy and is selective for B cells, so inhibition leads to a B-cell–specific phenotype

141
Q

Antiapoptotic therapies for CLL target

A

BCL2

142
Q

Following the completion of therapy or during therapy for indefinite targeted agents in CLL, response is initially assessed using

A

PE and laboratory studies

If residual disease is not detected using these methodologies, CT scans are used to assess response

Bone marrow biopsies with flow cytometry are indicated if no disease is detected to confirm CR

143
Q

In CLL, if no malignant cells can be detected in the bone marrow down to a level of______, the patient is said to be negative for minimal residual disease (MRD)

A

1 CLL cell in 10^4 leukocytes (0.01%)

144
Q

CLL Rai stage if lymphocytosis only

A

Low risk (stage 0)

145
Q

CLL Rai stage if lymphocytosis with lymphadenopathy, with or without splenomegaly or hepatomegaly

A

Intermediate risk (stage I/II)

146
Q

CLL Rai stage if lymphocytosis with anemia or thrombocytopenia due to bone marrow involvement

A

High risk (stage III/IV)

147
Q

CLL Binet stage A is ____ areas of lymphadenopathy

A

<3

148
Q

CLL Binet stage B is ____ areas of lymphadenopathy

A

≥3

149
Q

CLL Binet stage C is Hgb of ____ and Platelt of ____

A

≤ 10 g/dL

< 100,000/uL

150
Q

Symptoms Indicating Need for Therapy in CLL (5)

A
  1. Evidence of progressive marrow failure (worsening of anemia or thrombocytopenia not due to autoimmune destruction)
  2. Massive (≥6 cm below costal margin), progressive, or symptomatic splenomegaly
  3. Massive (≥10 cm), progressive, or symptomatic lymphadenopathy
  4. Autoimmune anemia or thrombocytopenia not responsive to standard therapy
  5. Constitutional symptoms (one or more of the following: unintentional weight loss ≥10% over 6 months, significant fatigue, fevers ≥100.5°C for 2+ weeks without infection, night sweats for >1 month without infection)
151
Q

Complete response in CLL therapy in terms of

Lymphocyte count
Lymph nodes
Spleen/Liver size
Bone marrow
PBS counts
A

Lymphocyte count: <4000/μL

Lymph nodes: None >1.5 cm

Spleen/Liver size: Not palpable

Bone marrow: Normocellular, <30% lymphocytes, no B lymphoid nodules

PBS counts: Platelet count >100,000/μL, Hemoglobin >11 g/dL, Neutrophils >1500/μL

152
Q

CML is the consequence of the balanced translocation between chromosomes

A

9 and 22 (t[9;22][q34;11])

153
Q

CNL has been associated with a translocation of chromosomes

A

t(15;19) translocation

154
Q

CEL occurs with a deletion or balanced translocations involving what gene

A

PDGFRa gene

155
Q

Myeloproliferative disorders that are characterized by driver mutations that directly or indirectly constitutively activate JAK2, a tyrosine kinase essential for the function of the erythropoietin and thrombopoietin receptors and also utilized by the granulocyte colony-stimulating factor receptor

A 
polycythemia vera (PV)
primary myelofibrosis (PMF)
essential thrombocytosis (ET)
156
Q

A clonal hematopoietic stem cell disorder in which phenotypically normal red cells, granulocytes, and platelets accumulate in the absence of a recognizable physiologic stimulus

A

Polycythemia vera

157
Q

The most common of the myeloproliferative neoplasms

A

Polycythemia vera

158
Q

A mutation in the autoinhibitory pseudokinase domain of the tyrosine kinase JAK2 that replaces valine with phenylalanine (V617F), causing constitutive kinase activation—appears to have a central role in the pathogenesis of

A

Polycythemia vera

159
Q

A member of an evolutionarily well-conserved, nonreceptor tyrosine kinase family and serves as the cognate tyrosine kinase for the erythropoietin and thrombopoietin receptors.

A

JAK2

160
Q

most common cytogenetic abnormality in PV

A

heterozygosity on chromosome 9p involving the segment containing the JAK2 locus over time due to mitotic recombination (uniparental disomy)

161
Q

Homozygosity for JAK2 V617F is expressed most frequently in

A

PV (60%)

lesser extent in PMF (50%) but is rare in ET

162
Q

Cytogenetic abnormality that is the basis for many of the phenotypic and biochemical characteristics of PV such as increased blood cell production and increased inflammatory cytokine production

A

JAK2 V617F

However, it cannot solely account for the entire PV phenotype and is probably not the initiating lesion in any of the MPN

163
Q

Symptom that distinguish PV from other causes of erythrocytosis

A

aquagenic pruritus

164
Q

Hepatic venous thrombosis is also known as

A

Budd-Chiari syndrome

PV should be suspected in any patient who develops hepatic vein thrombosis

165
Q

Erythema, burning, and pain in the extremities, a symptom complex known as________, is a complication of thrombocytosis in PV due to increased platelet stickiness.

A

erythromelalgia

166
Q

Unless the hemoglobin level is _____(hematocrit_____), it is not possible to distinguish true erythrocytosis from disorders causing plasma volume contraction.

A

≥20 g/dL

≥60%

167
Q

Relative erythrocytosis due to a reduction in plasma volume alone is known as

A

stress or spurious erythrocytosis or Gaisböck’s syndrome

168
Q

Only three situations cause microcytic erythrocytosis:

A

β-thalassemia trait
Hypoxic erythrocytosis
PV

169
Q

The assay for _______ has superseded other tests for establishing the diagnosis of PV.

A

JAK2 V617F

170
Q

T or F. A bone marrow aspirate and biopsy of PV provide no specific diagnostic information because these may be normal or indistinguishable from ET or PMF

A

True

171
Q

The most significant complication and often the presenting manifestation of PV

A

Thrombosis due to erythrocytosis

172
Q

In PV, maintenance of the hemoglobin level at ______ and hematocrit _____ in men and hematocrit ____ in women is mandatory to avoid thrombotic complications.

A

≤140 g/L (14 g/dL)
<45%

≤120 g/L (12 g/dL)
<42%

173
Q

In most PV patients, once an iron-deficient state is achieved, phlebotomy is usually only required _____ intervals.

A

at 3-month

174
Q

T or F. Neither thrombocytosis nor leukocytosis are correlated with thrombosis in PV, in contrast to the strong correlation between erythrocytosis and thrombosis.

A

True

175
Q

T or F. The use of salicylates is given to prevent thrombosis in PV patients.

A

False. It is not only potentially harmful if the red cell mass is not controlled by phlebotomy, but is also an unproven remedy.

176
Q

Given in PV when chemotherapy is used to avoid further elevation of the uric acid

A

Allopurinol

177
Q

Treatment of generalized pruritus in PV (4)

A

JAK1/2 inhibitor, ruxolitinib
pegylated interferon α (IFN-α)
psoralens with ultraviolet light in the A range (PUVA) therapy
hydroxyurea

Intractable to antihistamines or antidepressants such as doxepin and can be a major problem in PV

178
Q

Managemnt of symptomatic splenomegaly in PV (2)

A

Ruxolitinib

Pegylated IFN-α

179
Q

A phosphodiesterase inhibitor that can reduce the platelet count and, if tolerated, is preferable to hydroxyurea because it lacks marrow toxicity and is also protective against venous thrombosis while hydroxyurea is not

A

Anagrelide

180
Q

Alkylating agents and radioactive sodium phosphate are avoided in PV because they are

A

leukemogenic in PV

181
Q

Preferred cytotoxic agent in PV

A

Hydroxyurea

But this drug does not prevent either thrombosis or myelofibrosis in PV, is itself leukemogenic, and should be used for as short a time as possible

182
Q

JAK2 inhibitor used in PV

A

Ruxolitinib

183
Q

Chronic PMF (other designations include idiopathic myelofibrosis, agnogenic myeloid metaplasia, or myelofibrosis with myeloid metaplasia) is a clonal hematopoietic stem cell disorder associated with mutations in (3)

A

JAK2
MPL
CALR

184
Q

Chronic primary myelofibrosis is characterized by (3)

A

marrow fibrosis
extramedullary hematopoiesis
splenomegaly

185
Q

Least common myeloproliferative neoplasm

A

Primary myelofibrosis

186
Q

Myeloproliferative neoplasms that present with myelofibrosis (3)

A

Polycythemia vera
CML
Primary myelofibrosis

187
Q

Primary myelofibrosis primarily afflicts

A

men in their sixth decade or later

188
Q

T or F. BMA is the diagnostic test to detect myelofibrosis in PMF.

A

False. Marrow is usually inaspirable due to the myelofibrosis. But marrow biopsy will reveal a hypercellular marrow with trilineage hyperplasia and, in particular, increased numbers of megakaryocytes in clusters and with large, dysplastic nuclei

189
Q

T or F. PMF is a diagnosis of exclusion

A

True.

190
Q

Management of PMF

A

glucocorticoids + low-dose thalidomide
Roxulitinib
Allogeneic bone marrow transplantation

191
Q

The most distressing and intractable problem for PMF patients

A

Splenomegaly

Causing abdominal pain, portal hypertension, easy satiety, and cachexia

Surgical removal of a massive spleen is associated with significant postoperative complications including mesenteric venous thrombosis, hemorrhage, rebound leukocytosis and thrombocytosis, and hepatic extramedullary hematopoiesis with no amelioration of either anemia or thrombocytopenia when present. For unexplained reasons, splenectomy also increases the risk of blastic transformation.

192
Q

The only curative treatment for PMF and should be considered in younger patients and older patients with high risk disease

A

Allogeneic bone marrow transplantation

193
Q

ET (other designations include essential thrombocythemia, idiopathic thrombocytosis, primary thrombocytosis, and hemorrhagic thrombocythemia) is a clonal hematopoietic stem cell disorder associated with mutations in (3)

A

JAK2 (V617F)
MPL
CALR

194
Q

Essential thrombocytosis sex predilection

A

Female

195
Q

Usual PE in essential thrombocytosis

A

Usually unremarkable except for mild splenomegaly

Significant splenomegaly is indicative of another MPN, in particular PV, PMF, or CML.

196
Q

In essential thrombocytosis, this electrolyte abnormality is common but is a test tube artifact only

A

Hyperkalemia
The large mass of circulating platelets may prevent the accurate measurement of serum potassium due to release of platelet potassium upon blood clotting

197
Q

Absent marrow iron in the presence of marrow hypercellularity is a feature of

A

PV

198
Q

Appears to be the most important risk factor for thrombosis in ET patients

A

tobacco use

199
Q

Very high platelet counts are associated primarily with hemorrhage due to acquired

A

von Willebrand’s disease

PSBIM (12 decks)

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