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ASH Image Bank (2004); doi:10.1182/ashimagebank-2004-101098
Copyright © 2004 by the American Society of Hematology.
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Full Case Study

Plasma Cell Leukemia

Tsuyako Saito

Isao Miyoshi

Hirokuni Taguchi

Kochi Medical School



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  		Figure 1. Bone marrow smear showing numerous myeloma cells with an eccentrically placed nucleus and perinuclear halo.

 


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  		Figure 2. Peripheral blood smear showing five myeloma cells along with rouleaux formation of erythrocytes.

 


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  		Figure 3. Section of the liver showing infiltration of myeloma cells in the portal space and sinusoids.

 


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  		Figure 4. Section of the kidney showing interstitial infiltration of myeloma cells, hyaline casts in the tubules, and degenerative changes of the tabular epithelia.

 

   Clinical Summary
TOP
 Clinical Summary
Diagnosis
 Discussion
 Differential Diagnosis
 
A 64-year-old woman was admitted for anemia and lumbago.  Physical examination was unremarkable except for conjunctival pallor.  Radiograghs demonstrated punched-out lesions in the skull and compression fracture of a lumbar vertebra.  The erythrocyte sedimentation rate was 172 mm/hour.  The erythrocyte count was 182x104/mL, hematocrit 19%, hemoglobin 5.9 g/dL, leukocyte count 9000/mL with a normal differential, and platelet count was 136 000/mL.  The total protein was 14.7 g/dL with albumin of 2.4 g/dL and globulin of 12.3 g/dL.  There was a monoclonal spike on serum protein electrophoresis.  Immunoelectrophoresis determined the paraprotein to be IgG lambda.  The serum IgG was 8133 mg/dL, IgA 18 mg/dL, and IgM 5 mg/dL.  The urine was positive for Bence Jone lambda protein.  The serum ALT, LDH, BUN, and uric acid were slightly elevated.  A bone marrow aspirate revealed a nucleated cell count of 64 000/mL with 66% myeloma cells (Figure 1).  The patient was treated with a combination of vincristine, doxorubicin, and dexamethasone.  After an episode of pneumonia was overcome, this regimen was switched to melphalan and prednisolone, and she was discharged with some response.  However, 8.5 months after diagnosis, she was readmitted for severe backache.  At this time, the leukocyte count was 13 500/mL with 15% myeloma cells and a few immature myeloid forms and erythroblasts.  There was progression of thrombocytopenia and renal insufficiency.  Terminally, the leukocyte count was 10 600/mL with 81% myeloma cells (Figure 2).  The patient soon developed bloody stool and Staphylococcus aureus septicemia, and died on day 9.  Postmortem examination revealed infiltration of myeloma cells in the liver (Figure 3), spleen, kidneys (Figure 4), lungs, and lymph nodes in the hepatic hilum and around the abdominal aorta.  The bone marrow was diffusely packed with myeloma cells to the complete exclusion of fat cells.  Extramedullary hematopoiesis was found in the liver and spleen.  The kidneys showed features of "myeloma kidney."  There were multiple erosions in the stomach and descending colon.  An incidental finding was bronchioloalveolar cell carcinoma, 2x2 cm in size, in the right upper lung.  There was no deposition of amyloid in any organs examined. 

Sex
Female

Age
64

Ethnicity
Japanese


   Diagnosis
TOP
 Clinical Summary
 Diagnosis
Discussion
 Differential Diagnosis
 
Plasma Cell Leukemia


   Discussion
TOP
 Clinical Summary
 Diagnosis
 Discussion
Differential Diagnosis
 
Plasma cell leukemia is classified into two types, primary and secondary.  Primary plasma cell leukemia develops without preceding multiple myeloma, whereas secondary plasma cell leukemia evolves as a late leukemic stage of multiple myeloma.  It is reported that patients with primary plasma cell leukemia have a higher incidence of organomegaly and respond better to chemotherapy than do patients with secondary plasma cell leukemia.  The prognosis of our patient was poor, because she presented with a high tumor burden as shown by an IgG spike greater than 7 g/dL and hematocrit less than 25%, although there was no hypercalcemia.  In addition, infectious complication and renal damage prohibited the use of intensive chemotherapy.  Eventually, her IgG myeloma progressed to plasma cell leukemia that involved many organs.  This situation is analogous to leukemic conversion of malignant lymphoma.  It was rather unexpected to see extramedullary hematopoiesis in the liver and spleen of our patient.  At the time of the second admission, her peripheral blood exhibited leukoerythroblastosis as well as many myeloma cells.  It is likely that this leukoerythroblastic blood picture was caused by the extramedullary hematopoiesis. 


   Differential Diagnosis
TOP
 Clinical Summary
 Diagnosis
 Discussion
 Differential Diagnosis
 
Chronic lymphocytic leukemia, Malignant lymphoma, Waldenström’s macroglobulinemia


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Related ASH-SAP Chapter:space logo
Chapter 13: Plasma cell dyscrasias

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Copyright © 2004 by the American Society of Hematology.