Chronic Lymphoproliferative Disorders

Question 1

Outline the clinical features and laboratory findings specific to hairy cell leukaemia (5)

Answer 1

Rare, mature B-cell neoplasm which occurs predominantly in middle-aged adults (40-60 years). Males are affected more than females. Patients typically have splenomegaly and pancytopenia with no lymphadenopathy. Recurrent infections are a common presenting feature. Medium-sized lymphoid cells with abundant cytoplasm with “hairy” projections (hairy cells) are seen on the peripheral blood smear

Question 2

PCM is a neoplasm of terminally differentiated immunoglobulin-secreting B-cells. List three (3) tests that are useful in the diagnosis of this malignancy (1x3 = 3)

Answer 2

Protein electrophoresis (serum/ urine); u-BJP; SFLC assay; bone marrow biopsy

Question 3

List four (4) common clinical features with which a patient with PCM presents (2)

Answer 3

Bone pain/ Back pain, Fractures, Anaemia, Hypercalcaemia symptoms, Recurrent infections

Question 4

List three (3) tests that detect the complications of PCM (name the test as well as possible findings) (2x3 = 6)

Answer 4

FBC (Anaemia, neutropenia/ thrombocytopenia later in disease)
Renal function tests (Renal failure)
Calcium level (Hypercalcaemia)
Skeletal survey (Osteopenia, lytic lesions)
Specific XR (Pathological fractures)

Question 5

A 65-year-old woman presents with severe lower back pain. Her Hb is 8.5g/dl (reference range 12-16g/dl). Multiple myeloma Dx.

List four (4) contributing factors to the development of anaemia in this disorder (½x4 = 2)

Answer 5

Anaemia of chronic disorders, Renal failure, Bone marrow infiltration, Bleeding (paraprotein interferes with platelet function)

Question 6

List three (3) investigations you would perform to confirm the diagnosis of multiple myeloma (1½)

Answer 6

SPEP (monoclonal peak), UBJ protein urea, Bone marrow clonal plasma cell

Question 7

State what would you expect the Erythrocyte Sedimentation Rate to be in multiple myeloma (½)

Answer 7

Markedly elevated

Question 8

A 40-year-old woman presents with abdominal swelling fatigue, loss of weight, night sweats and easy bruising. A full blood count = WCC 15 (4-10x10^9/L) Hb 8 (12-15g/dl) PLT 20 (170-400x10^9/L). The smear shows the presence of circulating abnormal cells identified as Burkitt’s lymphoma cells. She has also tested HIV positive with a CD4 count of 205.

Of the three clinical variants of Burkitt’s lymphoma, state which variant is likely to occur in this patient (1)

Answer 8

Immunodeficiency-associated Burkitt’s lymphoma

Question 9

Name the cytogenetic abnormality which is common in Burkitt’s lymphoma (1) (NB)

Answer 9

t(8;14) – c-myc rearrangement

Question 10

List four (4) poor prognostic features in Burkitt’s lymphoma (2)

Answer 10

Bone marrow involvement, CNS involvement, tumour size >10cm, raised serum LDH