Lymphoid neoplasms and plasma cell disorders

14 DOH Abu Dhabi (HAAD) lab licensing practice questions on Lymphoid neoplasms and plasma cell disorders, part of Hematology. The first 14 are below; the first 5 with answers and explanations (tap Show answer after you have chosen), the rest in Learn with a free account.

Question 1 · Hard
A 64-year-old man has night sweats, a spleen 8 cm below the costal margin, small red skin papules and a pleural effusion. Results: WBC 186 × 10^9/L with 92% lymphocytes, Hb 102 g/L (10.2 g/dL), platelets 74 × 10^9/L and LD three times the upper reference limit. The film shows medium-sized lymphocytes with round to irregular nuclei, a single visible nucleolus and deeply basophilic cytoplasm with small surface blebs. Flow cytometry: CD2+, CD3 weak, CD5+, CD7 bright, CD4 and CD8 co-expressed, CD25−, TdT−, CD1a− and CD19−. HTLV-1 serology is negative. The most likely diagnosis is:
1. AT-lymphoblastic leukemia
2. BAdult T-cell leukemia/lymphoma
3. CT-prolymphocytic leukemia
4. DSézary syndrome
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Answer: C · T-prolymphocytic leukemia
A very high lymphocyte count with splenomegaly, skin lesions and a serous effusion, and medium-sized cells that have a visible nucleolus and cytoplasmic blebs, is the classic picture of T-prolymphocytic leukemia. The mature, TdT-negative T-cell phenotype with bright CD7 and CD4/CD8 co-expression supports it, and most cases carry an inv(14) or t(14;14) that activates TCL1A. Sézary cells have cerebriform nuclei and usually lose CD7. Adult T-cell leukemia/lymphoma has flower-shaped nuclei, is CD25+ and is caused by HTLV-1, which is negative here. T-lymphoblastic leukemia cells are TdT+, often CD1a+, and have fine blast chromatin.
- A: Lymphoblasts are TdT+ and often CD1a+; these cells are TdT− and CD1a−.
- B: ATLL cells are flower-shaped and CD25+, and HTLV-1 is negative here.
- D: Sézary cells are cerebriform and usually CD7 negative.
Question 2 · Hard
A 66-year-old has an IgG kappa paraprotein of 36 g/L (3.6 g/dL) and 18% clonal plasma cells in the marrow. Hemoglobin, calcium and creatinine are normal, whole-body MRI shows no lesions, and the involved to uninvolved free light chain ratio is 14. Under the IMWG criteria, which diagnosis fits?
1. AActive multiple myeloma
2. BSolitary plasmacytoma of bone
3. CMonoclonal gammopathy (MGUS)
4. DSmoldering multiple myeloma
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Answer: D · Smoldering multiple myeloma
Smoldering myeloma needs a serum M-protein of 30 g/L or more and/or 10 to 59% clonal marrow plasma cells, with no myeloma-defining event. This patient meets both thresholds but has no CRAB feature, fewer than 60% plasma cells, a light chain ratio below 100 and no MRI lesions. MGUS would need an M-protein below 30 g/L and fewer than 10% plasma cells. Smoldering myeloma is monitored closely because it progresses to active myeloma at about 10% a year in the first 5 years.
- A: Active myeloma needs a myeloma-defining event, and none is present.
- B: A solitary plasmacytoma is a single lesion with little or no marrow plasmacytosis.
- C: MGUS requires an M-protein below 30 g/L and fewer than 10% plasma cells.
Question 3 · Hard
A 50-year-old man has skin nodules, a corrected calcium of 3.3 mmol/L (13.2 mg/dL; reference 2.15 to 2.55 mmol/L) and WBC 48 × 10^9/L. The film shows lymphocytes with multilobed, flower-shaped nuclei. Flow cytometry: CD3+, CD4+, CD25+, CD7−. The most likely diagnosis is:
1. AAdult T-cell leukemia/lymphoma
2. BHairy cell leukemia
3. CT-cell prolymphocytic leukemia
4. DSézary syndrome
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Answer: A · Adult T-cell leukemia/lymphoma
Adult T-cell leukemia/lymphoma is caused by HTLV-1 and is a neoplasm of CD4-positive T cells that strongly express CD25. Multilobed flower cells, hypercalcemia from tumor-produced factors such as PTHrP, and skin lesions are typical. HTLV-1 serology confirms the link; the virus is endemic in southwestern Japan, the Caribbean and parts of Africa and South America. The acute form has a poor prognosis.
- B: Hairy cell leukemia is a B-cell neoplasm with pancytopenia and CD103.
- C: T-cell prolymphocytic leukemia cells usually keep strong CD7 and have a prominent nucleolus.
- D: Sézary syndrome presents with erythroderma and cerebriform cells, and hypercalcemia is not typical.
Question 4 · Easy
In multiple myeloma, the most common class of serum paraprotein is:
1. AIgM
2. BIgD
3. CIgA
4. DIgG
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Answer: D · IgG
About half of myelomas make an IgG paraprotein and about a fifth make IgA. Around 15 to 20% make light chains only, which can be missed on serum electrophoresis and need free light chain or urine testing. IgD myeloma is rare, and an IgM paraprotein usually points to Waldenström macroglobulinemia or IgM MGUS instead. The class is confirmed by immunofixation.
- A: An IgM paraprotein suggests Waldenström macroglobulinemia rather than myeloma.
- B: IgD myeloma is rare, about 1 to 2% of cases.
- C: IgA is the second most common, at about 20%.
Question 5 · Medium
Medium-sized blasts with deeply basophilic, heavily vacuolated cytoplasm are CD19+, CD10+, TdT negative and express surface IgM with lambda light chain restriction. The most likely diagnosis is:
1. ADiffuse large B-cell lymphoma
2. BB-lymphoblastic leukemia
3. CFollicular lymphoma
4. DBurkitt lymphoma
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Answer: D · Burkitt lymphoma
Burkitt cells are mature B cells, so they carry surface immunoglobulin with a single light chain and lack TdT and usually CD34. They are CD10 and BCL6 positive, BCL2 negative, and almost every cell is dividing (Ki-67 close to 100%). The lipid-filled vacuoles and deep blue cytoplasm are typical, and a MYC rearrangement, usually t(8;14), confirms the diagnosis. Telling it apart from B-lymphoblastic leukemia matters because the treatment differs.
- A: DLBCL cells are large, and this vacuolated, medium-sized picture is classic for Burkitt.
- B: B-lymphoblastic leukemia is TdT positive and usually lacks surface immunoglobulin.
- C: Follicular lymphoma cells are small cleaved cells, not vacuolated blasts.
Question 6 · Hard
A 72-year-old with CLL has a fall in hemoglobin from 128 to 84 g/L (12.8 to 8.4 g/dL) over 3 weeks. Reticulocytes are 9% (reference 0.5 to 2.5%), bilirubin and LD are raised, the film shows spherocytes, and the DAT is positive with anti-IgG. The most likely cause of the anemia is:
1. AWarm autoimmune hemolytic anemia
2. BCold agglutinin disease
3. CMarrow replacement by CLL cells
4. DPure red cell aplasia
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Question 7 · Hard
A 68-year-old with CLL, stable for 6 years, develops fevers, weight loss and one rapidly enlarging neck node. Serum LD is 3 times the upper reference limit. The node biopsy shows sheets of large B cells with a high Ki-67. This is most consistent with:
1. ATransformation to acute lymphoblastic leukemia
2. BReactive lymphadenitis from an infection
3. CRichter transformation to large B-cell lymphoma
4. DNodal progression of the original CLL
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Question 8 · Medium
Which antigen is typically positive in CLL but negative in most mantle cell lymphomas, helping to separate these two CD5-positive B-cell neoplasms?
1. ACD20
2. BCD19
3. CCD10
4. DCD200
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Question 9 · Hard
A 58-year-old with rheumatoid arthritis has recurrent infections. WBC is 6.8 × 10^9/L, with neutrophils 0.4 × 10^9/L and lymphocytes 5.6 × 10^9/L. Most lymphocytes are large, with abundant pale cytoplasm and coarse azurophilic granules. They are CD3+, CD8+, CD57+ and CD16+, and T-cell receptor gene studies show a clonal population. The most likely diagnosis is:
1. AT-cell large granular lymphocytic leukemia
2. BB-cell chronic lymphocytic leukemia
3. CReactive T-cell lymphocytosis after a virus
4. DSézary syndrome
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Question 10 · Medium
In CLL, which genetic finding predicts the poorest response to chemoimmunotherapy?
1. ADeletion of 17p (TP53)
2. BDeletion of 13q14 as the sole change
3. CTrisomy 12
4. DMutated IGHV genes
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Question 11 · Easy
Under the iwCLL criteria, chronic lymphocytic leukemia requires a sustained blood count of clonal B lymphocytes of at least:
1. A10 × 10^9/L
2. B4 × 10^9/L
3. C5 × 10^9/L
4. D1 × 10^9/L
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Question 12 · Medium
The Hodgkin Reed-Sternberg cells of classic Hodgkin lymphoma typically have which immunophenotype?
1. ACD30+, CD15+, CD45−, PAX5 weak
2. BCD20+, CD45+, CD30−, CD15−
3. CCD10+, BCL6+, CD20+, CD45+
4. DCD30+, ALK+, CD15−, CD20−
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Question 13 · Easy
Mott cells, plasma cells packed with grape-like globules, contain which inclusions?
1. ADutcher bodies
2. BDöhle bodies
3. CAuer rods
4. DRussell bodies
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Question 14 · Medium
Under the IMWG criteria, which finding counts as a myeloma-defining event even without hypercalcemia, renal failure, anemia or bone lesions?
1. ASerum M-protein of 25 g/L (2.5 g/dL)
2. BInvolved:uninvolved FLC ratio of 100 or more
3. CMarked rouleaux on the blood film
4. DClonal marrow plasma cells of 15%
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Lymphoid neoplasms and plasma cell disorders

A 66-year-old has an IgG kappa paraprotein of 36 g/L (3.6 g/dL) and 18% clonal plasma cells in the marrow. Hemoglobin, calcium and creatinine are normal, whole-body MRI shows no lesions, and the involved to uninvolved free light chain ratio is 14. Under the IMWG criteria, which diagnosis fits?

A 50-year-old man has skin nodules, a corrected calcium of 3.3 mmol/L (13.2 mg/dL; reference 2.15 to 2.55 mmol/L) and WBC 48 × 10^9/L. The film shows lymphocytes with multilobed, flower-shaped nuclei. Flow cytometry: CD3+, CD4+, CD25+, CD7−. The most likely diagnosis is:

In multiple myeloma, the most common class of serum paraprotein is:

Medium-sized blasts with deeply basophilic, heavily vacuolated cytoplasm are CD19+, CD10+, TdT negative and express surface IgM with lambda light chain restriction. The most likely diagnosis is:

A 72-year-old with CLL has a fall in hemoglobin from 128 to 84 g/L (12.8 to 8.4 g/dL) over 3 weeks. Reticulocytes are 9% (reference 0.5 to 2.5%), bilirubin and LD are raised, the film shows spherocytes, and the DAT is positive with anti-IgG. The most likely cause of the anemia is:

A 68-year-old with CLL, stable for 6 years, develops fevers, weight loss and one rapidly enlarging neck node. Serum LD is 3 times the upper reference limit. The node biopsy shows sheets of large B cells with a high Ki-67. This is most consistent with:

Which antigen is typically positive in CLL but negative in most mantle cell lymphomas, helping to separate these two CD5-positive B-cell neoplasms?

In CLL, which genetic finding predicts the poorest response to chemoimmunotherapy?

Under the iwCLL criteria, chronic lymphocytic leukemia requires a sustained blood count of clonal B lymphocytes of at least:

The Hodgkin Reed-Sternberg cells of classic Hodgkin lymphoma typically have which immunophenotype?

Mott cells, plasma cells packed with grape-like globules, contain which inclusions?

Under the IMWG criteria, which finding counts as a myeloma-defining event even without hypercalcemia, renal failure, anemia or bone lesions?