Final Diagnosis -- IgD/Lambda Multiple Myeloma with Plasma Cell Leukemia

FINAL DIAGNOSIS:

IGD/LAMBDA MULTIPLE MYELOMA

DISCUSSION:

Case findings:

• Anemia, thrombocytopenia, Hypercalcemia and hyperuricemia were secondary to MM.
  
• Acute renal Failure was due to uric acid nephropathy and/or free lambda light chain.
  
• Acute respiratory distress was initially due to pulmonary edema secondary to volume overload which then proceed into ARDS.
  
• The acuteness of the signs and symptoms and the presence at diagnosis of anemia, thrombocytopenia, hypercalcemia and renal failure with in marked involvement of peripheral blood with myeloma cells suggest (Plasma Cell Leukemia).

Prevalence:

• IgD Multiple myeloma is a rare disease representing (0.4% - 2%) of all MM [1,6].

Case Reports:

• The largest series was 165 cases from Japan [3]
  
• The second largest series was 53 cases from Mayo Clinics [4]
  
• The third largest series was 28 from Southern France [5]

Geographic Distribution:

• Most of the cases were reported from Japan [3]

Bone Marrow Findings:

• No characteristic histological or BM features of IgD MM or plasmacytoma distinguishing them from other types.

Protein Electrophoresis:
• M-spike is present in (60%) of the cases,[4] usually in Gamma 1 or Beta regions.
  
• The remaining have either hypogammaglobulinemia or normal pattern.[4]
  
• Bence Jones proteinuria is present in (96%) of the cases.[4]
  
• Light chain is mostly Lambda (60%) followed by Kappa (38%) and indeterminate in (2%).[4]

Cytogentics:

• IgD MM is reported to be associated with: Complex hypodiploid karyotype, loss of Ch X, monosomy 13, several deletions of Ch (1,6,11,12), Translocations of Ch (4,9,10,15,16,21).[2]

Other Tests:

• Hyperamylasemia and increased Neuron specific enolase (NSE) were reported with IgD MM.[9]

Clinical Findings:

• The main presenting features were bone pain (72%), fatigue (36%), renal function impairment (33%), weight loss (32%), hypercalcemia (22%), extramedullary plasmacytoma (19%) and secondary amyloidosis (19%).[4]
  
• The majority of cases of IgD paraprotememia are MM witch may develop secondary plasmacytoma in (19%), a minority present with plasmacytoma or plasma cell leukemia.[6]
  
• IgD MGUS was reported.[7]

Survival Rate:

• The median duration of survival in IgD MM is shorter than in patients with other types of MM.[4]
  
• Median survival was calculated to be 11 months compared to 29 months in other types of MM.[5]

REFERENCES

1. Blade J. et al; Nonsecretory myeloma, IgD myeloma, and plasma cell leukemia; Hematology-Oncology Clinics of North America. 13(6):1259-72/1999 Dec.
2. Weinlander G. et al; Cytogenetic analysis and FISH in a case of IgD MM; Cancer Genitics and Cytogenetics. 105(2):172-6, 1998 Sep.
3. Shimamoto Y. et al; A new risk for grouping for IgD myeloma based on analysis of 165 Japanese patients; European Journal of Hematology. 47(4):262-7, 1991 Oct.
4. Blade J. et al; IgD MM: Presenting features, response to therapy, and survival in a series of 53 cases; Journal of Clinical Oncology.12(11):2398-404, 1994 Nov.
5. Le Quellec A et al; IgD MM. A retrospective study in the Languedoc region; Press Medicale. 18(22):1110-3, 1989 Jun 3.
6. Tichy M. et al; IgD paraproteinemias; Neoplasea.40(1):59-61, 1993.
7. O'Connor ML et al; Immunoglobulin D benign monoclonal gammopathy. A case report; Cancer. 68(3):611-6, 1991 Aug 1.
8. Delannoy A; Amylase-producing IgD-type multiple myeloma. [Review] [8 refs] Journal of Internal Medicine. 232(5):457-60, 1992 Nov.
9. Nakajima T.; Neuron specific enolase-producing IgD multiple myeloma with high serum amylase activity Rinsho Ketsueki - Japanese Journal of Clinical Hematology. 36(4):359-64, 1995 Apr.

Contributed by Hazim Najjar, MB, BS, MD