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Heme Board Review Question Notes > Hemepath Missed Questions > Flashcards

Hemepath Missed Questions Flashcards

1
Q

What immunophenotype in B-ALL indicates a possible MLL gene rearrangement ?

A

CD10 negative and CD15 positive
Infantile acute lymphoblastic leukemia
Translocation (4;11) usually
Associated with Topoisomerase inhibitor use

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2
Q

What findings are usually absent in cases of Toxoplasmosis?

A

Neutrophils, eosinophils and areas of necrosis

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3
Q

What is the most common secondary cytogenetic abnormality in Burkitt lymphoma that may indicate disease progression?

A

Duplication of 1q
- other abnormalities include trisomies of 7 and 12

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4
Q

Cyclin D1 is what type of gene?

A

Cell cycle regulator

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5
Q

What monoclonal proteins are more often associated with plasma cell leukemia ?

A
  • light chain only disease
  • IgD
  • IgE
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6
Q

What immunophenotypic markers are almost never seen in pure myeloid lineage leukemias ?

A
  • CD79a, CD10, CD2, and CD3
  • IMP: CD117 is most specific (other than MPO) for myeloid lineage as compared to CD13 and CD33
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7
Q

What immunophenotype hints at a B-ALL with t(1;19) ?

A
  • positive for CD19, CD10 and CD9
  • negative for CD34 and CD20
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8
Q

What are the chromosomes involved in the BCL6-IGH gene rearrangement seen in high grade or DLBCL?

A
  • t(3;14)
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9
Q

What is often seen in lymph nodes involved by Rosai Dorfmann disease ?

A
  • plasmacytosis, polyclonal hypergammaglobulinemia, and occasional Russel bodies
  • the histiocytes of Rosai Dorfmann often aberrantly express CD31
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10
Q

What type of molecule is CD31 and what cells normally express it ?

A
  • CD31 is a cell adhesion molecule
  • often expressed by endothelial cells, platelets, and granulocytes
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11
Q

What clinical features are seen in Pelger-Huet anomaly?

A
  • developmental delay
  • epilepsy
  • skeletal abnormalities
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12
Q

What clinical features are seen in myelokathexis ?

A
  • bacterial and fungal infections
  • very abnormal, hypersegmented neutrophils
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13
Q

What percentage of DLBCL have the BCL2 gene rearrangement [t(14;18)] ?

A
  • 20-30%
  • BCL6 aberrations on chromosome 3 are the most common alterations (30% of cases)
  • only 10% of DLBCL have cMYC rearranged, while nearly 100% of BL have it
    * of note, if MYC is rearranged, other complex cytogenetic findings are seen in DLBCL but not BL
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14
Q

What can typically be seen in the bone marrow of patient’s with plasma cell myeloma?

A
  • extensive fibrosis
  • consider myeloma in the d/d of dry tap bone marrows
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15
Q

What immunophenotype can be seen in ALCL ?

A
  • positive for: CD45, CD7, HLA-DR, CD13, CD16/56, CD25, CD30
  • negative for: CD43, CD3, TCR a/b, g/d
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16
Q

What T cell lymphomas are most common in the post-transplant setting ?

A
  • PTCL, NOS
  • Hepatosplenic T cell lymphoma
  • only ~15% of PTLDs and are usually EBV negative
  • worse prognosis than a B cell PTLD
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17
Q

The t(14;18) is specific to Follicular lymphoma, true or false ?

A
  • False
  • a subset of DLBCL can also harbor the translocation
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18
Q

In primary effusion lymphoma, it is thought that EBV plays what role?

A
  • EBV is a co-infection that occurs but is not the driving cause of the neoplasm
  • HHV8 is the driving infection
    * PEL can occur in non HIV patients, usually in areas of high infection with HHV8 (Mediterranean)
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19
Q

What is another type of lymphoma that can arise in the setting of HHV8 ?

A
  • Large B cell lymphoma arising from HHV8 positive multicentric Castlemann disease
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20
Q

Which hematopoietic malignancies are associated with hemophagocytosis ?

A
  • T cell lymphomas in particular- subcutaneous panniculitis like T cell lymphoma
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21
Q

What are independent risk

factors for CLL/SLL ?

A
  • >30% of cells expressing CD49d and CD38
  • these are independent poor prognostic risk factors
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22
Q

G-6-PD is part of which pathway?

A
  • enzyme in the pentose phosphate pathway
    • hexose mannose phosphate shunt
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23
Q

What is the most common enzyme

deficiency in hereditary erythrocyte disorders

of the glycolytic pathway?

A
  • pyruvate kinase
  • accounts for 90% of cases
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24
Q

What is the abnormal immunophenotype

for BPDCN ?

A
  • same immunophenotype as normal PDCs CD123, CD4, CD56
  • abnormal compared to PDCs
    • CD56
    • TdT (variable)
    • CD43
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25
Q

The presence of what mutation portends

a poor prognosis in AML s with t(8;21) and inv(16)?

A
  • KIT mutations

Note: the gene rearrangement de novo AMLs frequently have a normal karyotype.

Also, note, JAK2 mutations are not seen in de novo AML

26
Q

What are Dohle bodies composed of and what situations can they be seen in?

A
  • seen in high turnover rates of neutrophils, often more common in progranulocytic stage - composed of endoplasmic reticulum * they contain high RNA content so will be destroyed by ribonucleases - can be seen in normal pregnancy (who knew)
27
Q

What is a relatively specific sign for infection and/or acute alcohol poisoning on a peripheral smear ?

A
  • neutrophils with cytoplasmic vacuoles
28
Q

In what situations can you see

myeloid hyperplasia in the bone marrow ?

A
  • lithium therapy (shows maturation)
  • chronic infection
  • G-CSF therapy
  • MPN

Note: corticosteroids do not cause myeloid hyperplasia but rather cause peripheralization of the neutrophils (peripheral blood neutrophilia)

29
Q

In what situations can marginal zone hyperplasia

be seen in the spleen?

A
  • any time there is a sustained humoral reaction you can see this
    • ex: autoimmune disease
  • note the differences with marginal zone lymphoma
    • effacement of the germinal cener and mantle zone by small B cells
30
Q

What B cell lymphoma usually

demonstrates Trisomy 18 ?

A
  • MALT lymphoma
31
Q

t(11;18) is seen in which type of

MALT lymphomas ?

A
  • gastric
  • pulmonary
  • this is the API2 and MALT1 genes
32
Q

What abnormalities when present together

can indicate CMML with the right

morphologic context ?

A
  • TET2
  • SRSF2
  • these mutations are characteristic of CMML
33
Q

What type of cell is decreased in

anemia of chronic disease ?

A
  • siderocytes are decreased
    • these cells are normal erythroids with iron granules within their cytoplasm
    • these cells can incorporate iron into hemoglobin, unlike ring sideroblasts which cannot
  • note:
    • storage iron can be normal or increased
34
Q

What nonclonal conditions can

cause ring sideroblast formation?

A
  • hereditary sideroblastic anemia
  • alcoholism
  • lead poisoning
  • zinc toxicity
  • some antimicrobials such as INH and chloramphenicol therapy
35
Q

What is the morphology of

splenic peliosis ?

A
  • non-neoplastic blood filled cavities haphazardly scattered throughout the splenic red pulp
  • isolated splenic lesions in peliosis are rare
  • the blood filled spaces generally lack an endothelial cell lining
36
Q

What is unique about the immunophenotype

of Littoral cell angiomas ?

A
  • they express both endothelial and histiocytic markers (CD31, CD34, CD68, CD163)
  • this is a benign entity
37
Q

What is the most common tumor of the spleen ?

A
  • Hemangioma
    • must rule out angiosarcoma which should be atypical, have mitosis and show infiltrative growth
38
Q

What immunophenotype supports a lymphangioma

of the spleen ?

A
  • D2-40
  • also these can variably express other vascular markers such as CD31, CD34, Factor VIII
39
Q

What gene can be rearranged in a minority

of cases of MALT lymphoma ?

A
  • BCL10 gene at 1p22
40
Q

Review morphology of

Infectious mononucleosis

A
  • predominantly paracortical hyperplasia with some follicular hyperplasia
  • paracortex is moth eaten
  • polymorphous lymphocytic infiltrate is usually seen with scattered immunoblasts, sometimes the immunoblasts can form clusters or sheets and mimic DLBCL
41
Q

What is basophilic stippling and in what

conditions is it normally seen ?

A
  • denatured ribosomal RNA
  • seen in:
    • thalassemias
    • MDS
    • lead poisoning
42
Q

What are the features of

Congenital Dyserythropoietic Anemia Type I ?

A
  • negative RBC agglutination by anti-i and anti-I
  • negative serum acidified test (Ham Test)
  • Morphology:
    • erythroid precursors have thin chromatin bridges between erythroblasts
    • incompletely divided erythroid precursors
43
Q

What are the features of Congenital Dyserythropoietic Anemia

Type II ?

A
  • positive agglutination test by anti-i and anti-I
  • positive acidified serum test (Ham Test)
  • HEMPAS is a synonym for this disorder
    • Hemolytic anemia with a positive acidified serum acid test
  • Morphology
    • numerous binucleate and rare multinucleated polychromatic erythroblasts
44
Q

What are the features of Congenital Dyserythropoietic

Anemia Type III ?

A
  • negative for RBC agglutination with anti-i and anti-I
  • negative acidified serum test (Ham Test)
  • Morphology
    • giant, multinucleated erythroblasts

Note: there are 3 major types of CDA

45
Q

What is the expression of CD43

in Follicular Lymphoma?

A
  • negative for CD43

IMP: Mantle cell lymphoma, CLL and Marginal zone are often positive for CD43

46
Q

What is the clinical presentation

of Parvovirus B19?

A
  • called Fifth’s disease or erythrema infectiosum
  • viral prodrome with slapped cheek effectn and anemia
  • it can be associated with pure red cell aplasia
    • will see giant pronormoblasts with viral inclusions in the bone marrow
  • Parvovirus spreads:
    • respiratory secretions
    • transplacental (mom to baby)
    • blood transfusions
47
Q

What clinical features would

suggest Fanconi Anemia ?

A
  • presentation of bone marrow failure
  • abnormal skin pigmentation (cafe au lait)
  • skeletal abnormalities
  • mental retardation
  • short stature
  • renal abnormalities
  • IMP more common in:
    • Ashkenazi Jews
    • South Africans
48
Q

What is the genetic defect in

Fanconi anemia and its inheritance pattern?

A
  • disorder in 1 out of 15 genes important for DNA repair
  • causes DNA damage in hematopoietic and epithelial cells
  • most common inheritance pattern:
    • Autosomal recessive
    • less likely X-linked recessive
49
Q

How is Fanconi Anemia diagnosed

and what are these patients at risk for?

A
  • Diagnosis:
    • lymphocytes and fibroblasts are hypersensitive to DNA cross-linking agents such as mitomycin
    • positive chromosome breakage studies
  • At risk for:
    • aplastic anemia usually develops by age 10
    • if they survive this at risk for MDS, AML and other epithelial malignancies
50
Q

What are the findings in Wiskott Aldrich Syndrome ?

A
  • eczema
  • thrombocytopenia with small platelets
  • immune deficiency
51
Q

What are the findings of WHIM syndrome ?

A
  • Warts
  • Hypogammaglobulinemia
  • infections
  • Myelokathexis
    • causes retention of neutrophils in bone marrow

IMP: appears to only affect neutrophils

52
Q

What are the key findings in

Chediak Higashi Syndrome ?

A
  • microtubule polarization defect (LYST gene)
  • anemia, neutropenia, numerous infections (defect in phagocytosis)
  • platelet dysfunction (first wave only)
  • oculocutaneous albnism and neurologic defects
  • abnormally giant granules in granulocytes that range in color from gray to red
53
Q

What are features of dyskeratosis congenita?

A
  • defects in telomerase maintenance (multiple genes)
  • pleiotropic effects mimicking premature aging in multiple different organ systems
  • one or more hematopoietic lineages are affected
54
Q

What is the major feature of

Diamond Blackfan anemia ?

A
  • erythrocyte lineage is affected only
55
Q

What are the key features

of Schwachman Diamond Syndrome ?

A
  • defect in SBDS gene
  • exocrine pancreatic insufficiency
  • bone marrow dysfunction
  • skeletal abnormalities
  • short stature
56
Q

What are the key features of severe

congenital neutropenia?

A
  • mutations in ELA2 (neutrophil elastase)
  • mutations in SCN2 (cyclical neutropenia)
57
Q

On a CSF specimen how are

Medulloblastoma and ALL differentiated ?

A
  • Medulloblastoma can show nuclear molding while ALL should not
  • nucleoli in medulloblastoma can be inconspicuous or prominent
58
Q

What is the normal counterpart cell

to ALCL ?

A
  • ALK positive is derived from CD4 positive T cells with cytotoxic features
59
Q

Which lymphoma is characteristically derived

from NK cells or Cytotoxic T cells ?

A
  • Extranodal NK/T cell lymphoma
  • usually more NK cells but can be a subset from T cells
  • this lymphoma usually involves the nasal cavity but secondary lymph node involvement can be seen
60
Q

Which lymphoma is from

precursor Thymic T cells ?

A
  • T-ALL
61
Q

Which lymphoma is derived from gamma delta

T cells ?

A
  • hepatosplenic T cell lymphoma
  • certain GI lymphomas
  • primary cutaneous gamma delta T cell lymphoma

IMP: none of these lymphomas go to the lymph nodes

Heme Board Review Question Notes (2 decks)

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