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Heme Week 2 > Lymphoid Disorders II > Flashcards

Lymphoid Disorders II Flashcards

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1
Q

This is a neoplastic proliferation of plasma cells, usually with paraproteinemia

A

Plasma cell dyscrasias

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2
Q

Plasma cell dyscrasias is neoplastic proliferation of plasma cells, usually with this

A

Paraproteinemia

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3
Q

This form of Plasma cell dyscrasias has monoclonal protein <30 g/L, clonal plasma cells in marrow, and no myeloma defining events

A

Monoclonal gammopathy of undetermined significance

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4
Q

What is the treatment for Monoclonal gammopathy of undetermined significance?

A

No treatment; monitor for progression to myeloma, primary amyloidosis, or Waldenstrom

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5
Q

This is a proliferation of malignant (monoclonal) plasma cells with clinical manifestations (or meeting quantitative threshold)

A

Multiple myeloma

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6
Q

This is the 2nd most common hematologic malignancy

A

Multiple myeloma

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7
Q

Multiple myeloma always starts as this condition

A

Monoclonal gammopathy of undetermined significance

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8
Q

What is the diagnostic criteria for Multiple myeloma?

A

Bone marrow monoclonal plasma cells >10%
AND
At least one myeloma defining event (hypercalcemia, renal insufficiency, anemia, lytic bone lesions)

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9
Q

Lytic bone lesions, hypercalcemia, and anemia/cytopenia from marrow infiltration occur in this condition

A

Multiple myeloma

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10
Q

A 70 year old patient presents with back pain, and has signs of hyperviscosity and renal damage, likely due to this condition

A

Multiple myeloma

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11
Q

The majority of patients with Multiple myeloma present with this symptom

A

Back pain

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12
Q

Raynaud’s, deep vein thrombosis, impaired circulation, headache, visual disturbance, dyspnea, and heart failure occur as a result of this, which can occur in Multiple myeloma

A

Hyperviscosity syndrome

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13
Q

What are Bence-Jones proteins?

A

Free light chains that are produced by plasma cells in Multiple myeloma, and are filtered in the kidney, causing tubular damage

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14
Q

Bence-Jones proteins are most commonly seen in this condition

A

Multiple myeloma

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15
Q

These produce tubular damage in Multiple myeloma

A

Light chains

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16
Q

What produces glomerular damage in Multiple myeloma?

A

Amyloid deposits or non-amyloid proteins deposit

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17
Q

This molecule is increased with poor prognosis of Multiple myeloma
Reflects tumor burden

A

Beta-2 microglobulin

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18
Q

This is the light chain of MHC-I antigen with indicates poor prognosis of Multiple myeloma

A

Beta-2 microglobulin

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19
Q

Beta-2 microglobulin indicates poor prognosis of this neoplasm

A

Multiple myeloma

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20
Q

Peripheral smear shows rouleaux (stacked RBCs in coin-like pattern) in this condition

A

Multiple myeloma

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21
Q

This is a characteristic morphological finding in Multiple myeloma

A

Rouleaux
(stacked RBCs in a coin-like pattern)

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22
Q

This type of Plasma cell dyscrasias will have serum monoclonal IgG or IgA >30g/L, with urine monoclonal protein >500mg/24 hours
No myeloma defining events

A

Smoldering myeloma

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23
Q

In multiple myeloma, plasma cells may produce Ig of any isotype, but likely in this distribution

A

G > A > M > D > E

proportions same as benign plasma cells

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24
Q

This form of Plasma cell dyscrasias tends to be in younger patients, M protein in <30%, single bone or soft tissue lesion (often nasopharynx), excellent prognosis with XRT

A

Solitary plasmacytoma

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25
Solitary plasmacytoma is a single bone or soft tissue lesion, often in this location
Nasopharynx
26
This is a clinical syndrome of IgM secreting lymphoplasmacytic lymphoma
Waldenstrom macroglobulinemia
27
Waldenstrom macroglobulinemia involves secretion of this Ig
IgM
28
Does this describe Waldenstrom macroglobulinemia or IgM myeloma: Lytic lesions and hypercalcemia
IgM myeloma
29
Does this describe Waldenstrom macroglobulinemia or IgM myeloma: Hepatosplenomegaly and lymphadenopathy common
Waldenstrom macroglobulinemia
30
In Waldenstrom macroglobulinemia, do IgM light chains cause renal disease?
No, they are not filtered contrast to multiple myeloma, where light chains are produced in kidney and cause tubular damage
31
Waldenstrom macroglobulinemia symptoms are primarily due to this
Hyperviscosity syndrome
32
Waldenstrom macroglobulinemia frequently produces these, which are immunoglobulins that precipitate when cooled and dissolve when warmed
Cryoglobulins type I
33
This neoplasm will have symptoms of headache, visual disturbances, peripheral neuropathy, anemia (normo/normo), and cryoglobulinemia
Waldenstrom macroglobulinemia
34
These are immunoglobulins that precipitate when cooled and dissolve when warmed Produce symptoms in cold temps (acrocyanosis, Raynauds, vasculitis)
Cryoglobulins
35
Cryoglobulins in Waldenstrom macroglobulinemia are this type
Monoclonal IgM (type I)
36
The measurement of these may show response of Waldenstrom macroglobulinemia to therapy
Cryoglobulins
37
This type of cryoglobulin is mixed; monoclonal Ig (RF activity) and polyclonal IgG Seen in Hep C, other benign conditions
Type II
38
This type of cryoglobulin is polyclonal IgG and IgM, with RF activity Seen in viral infections, rheumatologic conditions
Type III
39
This condition is deposition of amyloid in non-CNS tissues
Systemic amyloidosis
40
This type of amyloid is composed of Ig light chains
AL
41
This type of amyloid is seen in plasma cell dyscrasia Usually in multiple myeloma, but may be in MGUS
AL
42
This type of amyloid is composed of serum amyloid A, a group of acute phase proteins
AA
43
This type of amyloid is seen in long-term chronic inflammatory states tuberculosis, rheumatoid arthritis
AA
44
Systemic AL amyloidosis involves deposition of this
Misfolded Ig light chains
45
Amyloid is positive in this stain
Congo red
46
Systemic AL amyloidosis is associated with this type of disorder
Clonal B cell or plasma cell disorder
47
This condition can result in neuropathy, renal disease, vasculopathy, and macroglossia
Systemic AL amyloidosis
48
This test is needed to confirm presence of amyloid in systemic AL amyloidosis
Tissue biopsy
49
In this neoplasm, small mature B cells are present in peripheral blood CD20+ CD5+ CD23+ monoclonal
Chronic lymphocytic leukemia
50
Chronic lymphocytic leukemia will have this immunophenotype
CD20+ CD5+ CD23+
51
Chronic lymphocytic leukemia is associated with this anemia
WAIHA warm autoimmune hemolytic anemia
52
Patients with Chronic lymphocytic leukemia have increased infections from this
Hypogammaglobulinemia
53
Chronic lymphocytic leukemia may undergo Richter's transformation to this
Large cell lymphoma
54
This is the lymph node form of Chronic lymphocytic leukemia
Small lymphocytic lymphoma
55
In this neoplasm, mature T cells are present in peripheral blood or nodes CD4+ T cells
Adult T cell leukemia/lymphoma
56
This type of T cells are present in peripheral blood or nodes in Adult T cell leukemia/lymphoma
CD4 T cells
57
Adult T cell leukemia/lymphoma is associated with this infection
HTLV
58
Lytic bone lesions and hypercalcemia, and clover cells, are characteristic of this neoplasm that is associated with HTLV infection
Adult T cell leukemia/lymphoma
59
In this neoplasm, mature B cells are present in peripheral blood, spleen, or bone marrow
Hairy cell leukemia
60
Hairy cell leukemia affects this patient population mostly
Middle age men avg age 54 yrs
61
Patients with this neoplasm present with infection, anemia, or bleeding often Cytopenias (including leukopenia) Monocytopenia is very common Splenomegaly Bone marrow fibrosis +/- dry tap
Hairy cell leukemia
62
This type of leukemia may produce bone marrow fibrosis and dry tap
Hairy cell leukemia
63
Cells in this neoplasm will show: CD20+ CD11c, CD103, CD25, CD123 TRAP stain on blood smear (Tartrate resistant acid phosphatase) ~100% with BRAF mutation
Hairy cell leukemia
64
Cells in Hairy cell leukemia will stain positive with this
TRAP Tartrate resistant acid phosphatase
65
Cells in this neoplasm stain positive on blood smear for TRAP (Tartrate resistant acid phosphatase)
Hairy cell leukemia
66
Near 100% of cells in Hairy cell leukemia will have this mutation
BRAF
67
Bone marrow infiltrate in this neoplasm is associated with "fried egg" cells, and may produce marrow fibrosis and dry tap
Hairy cell leukemia
68
Hairy cell leukemia involves this part of the spleen
Red pulp
69
Malignant neoplasms of immature lymphoid precursors (lymphoblasts) Most common malignancy of childhood Peak age: 3 years, but age spans into adulthood May be B or T cell lineage with corresponding lineage markers
Acute lymphoblastic leukemia
70
This is the most common malignancy of childhood
Acute lymphoblastic leukemia
71
This is the peak age of Acute lymphoblastic leukemia
3 years but age spans into adulthood
72
Does Acute lymphoblastic leukemia have a good or poor prognosis?
Excellent prognosis: 85% cure most cases are children most deaths are adults
73
This neoplasm will have highly proliferative lymphoblasts which fill marrow Presentation usually due to marrow expansion and cytopenias (bone pain, anemia, thrombocytopenia)
Acute lymphoblastic leukemia
74
A child who presents with bone pain, petechiae, ecchymoses, and pallor may have this neoplasm
Acute lymphoblastic leukemia
75
Is organ infiltration common in Acute lymphoblastic leukemia?
Yes bone pain, splenomegaly, thymic enlargement (vessel or airway compression), hepatomegaly/liver dysfunction, CNS (confusion, lethargy), testicular enlargement
76
Organ infiltration is common in this neoplasm that has a worse prognosis in adults, but is more common in children
Acute lymphoblastic leukemia
77
Do young or adult patients with Acute lymphoblastic leukemia have a better prognosis?
Young
78
This type of Acute lymphoblastic leukemia is more common in children
B-ALL
79
This type of Acute lymphoblastic leukemia is more common in adults
T-ALL
80
Children with Acute lymphoblastic leukemia often have this translocation
t(12;21)
81
Adults with Acute lymphoblastic leukemia often have this translocation
t(9;22)
82
Do children or adults with Acute lymphoblastic leukemia often have t(12;21)?
Children
83
Do children or adults with Acute lymphoblastic leukemia often have t(9;22)?
Adults
84
Do children or adults with Acute lymphoblastic leukemia more commonly have B cell type?
Children
85
Do children or adults with Acute lymphoblastic leukemia more commonly have T cell type?
Adults
86
These are 2 immaturity markers in Acute lymphoblastic leukemia
CD34 / TdT
87
Patients with this translocation have a poor prognosis with Acute lymphoblastic leukemia
t(9;22) more common in adults
88
Acute lymphoblastic leukemia has a poor prognosis with translocations in this gene
MLL (mixed lineage leukemia)
89
A patient with lytic bone lesions and a skin rash likely has this neoplasm
Adult T cell leukemia/lymphoma contrast to multiple myeloma, which would have lytic bone lesions but NO skin lesions

Heme Week 2 (7 decks)

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