Small Group Case # 8:
Thursday, September 19, 2002.
HEMATOLOGY/ONCOLOGY
1
HEMATOLOGY/ONCOLOGY
A 78 year-old-man presented to the emergency room for acute loss of vision in the left
eye. Ophthalmic examination in the E.R suggested retinal vein thrombosis. On further
evaluation patient described progressive tiredness and bone pain in the spine and hip. He
also described two back-to-back episodes of upper respiratory bacterial infections.
Physical examination revealed a pale white elderly man with purpura on the legs and
arms. No enlargement of the lymph nodes or spleen was noted.
A CBC showed the following values:
WBC
Neutrophil
Eosinophil
Basophil
Lymphocytes
Monocytes
= 7.8 x 109/L
= 63%
= 2%
=1
= 30%
= 4%
RBC
Hb/Hct
MCV
MCHC
MCH
Platelets
RDW
= 3.67 x 1012/L
= 9.8 g/dL / 24
= 89 fL
= 30 g/L
= 26.7 pg
= 352 x 109/L
= 14
Review of the peripheral blood smear showed pink, hyaline proteinaceous material and
normochromic normocytic red cells. No abnormal circulating cells were seen. The
reticulocyte count was 2%. A total serum protein was found to be 10.8 g/dl.
Q1. What other tests would you like to do at this point and why?
1. Serum and urine protein electrophoresis
2. Serum immunofixation
3. PT and PTT
4. Skeletal survey
Multiple small osteolytic lesions were found in the spine and hip bones. A serum protein
immunofixation after electrophoresis showed the following results:
Small Group Case # 8:
Thursday, September 19, 2002.
HEMATOLOGY/ONCOLOGY
2
Q2. What would you like to do next in your diagnostic evaluation and why?
Serum paraprotein quantitation, serum calcium, bone marrow biopsy and aspiration with
cytogenetics. Flow cytometry or immunohistochemistry if diagnosis is in question. Serum
-2 microglobulin and C-reactive protein for prognostication.
Examination of the bone marrow biopsy showed the following cells on an aspirate
Q3. Describe the complete immunophenotype and the best markers to identify these cells
CD138+, CD38+, best marker is cytoplasmic immunoglobulin light-chain
evaluation by immunohistochemistry or flow cytometry.
Other markers are CD56+, CD45-, CD20-, CD19-,
Q4. Describe this disease with respect to the following
1. General features
2. Various clinical presentations
3. Pathophysiology
4. Diagnostic criteria
5. Course
General features: Most common malignant plasma cell dyscrasia. A disease of adults
(usually > 60 years). Not seen in children. Refractory to treatment in most cases.
Small Group Case # 8:
Thursday, September 19, 2002.
3
HEMATOLOGY/ONCOLOGY
Various clinical presentations and pathophysiology:
Bone pain
BONE DESTRUCTION
Hypercalcemia
IL-1
IL-6
Release of cytokines
Anemia
Marrow infiltration
IL-6
Multiple myeloma
MONOCLONAL PROTEIN
IMMUNE DEFICIENCY
Renal failure
Amyloidosis
Hyperviscosity
Infection
Diagnostic criteria:
1 major + 1 minor criteria or 3 minor criteria
Major criteria:
1) Plasmacytoma on tissue biopsy
2) Marrow plasmacytosis (>30%)
3) Monoclonal protein
a) IgG >3.5 g/dl
b) IgA >2 g/dl
c) Light-chains only (Bence-Jones proteins) > 1 g/dl
Minor criteria:
1) Marrow plasmacytosis 10-29%
2) Monoclonal protein that is less than that required in major criteria
3) Lytic bone lesions
4) Decrease in uninvolved (non-clonal) immunoglobulins
a) IgM <50 mg/dl
b) IgA < 100 mg/dl
c) IgG < 600 mg/dl