POSTER 21
A DECADE OF LIFE WITH DIFFERENT FACES OF THE SAME DISEASE IN A DEVELOPING
COUNTRY
Maduwanthi Jayaweera1, M N Dilhani2 and Senani Williams3
(1)Dr., Colombo, Sri Lanka
(2)1 Senior Registrar in Clinical Haematology, Department of Pathology, Faculty of Medicine, University
of Kelaniya, Colombo, Sri Lanka
(3)Professor of Hematology, Department of Pathology, Faculty of Medicine, University of Kelaniya,
Colombo, Sri Lanka
Introduction:
Multiple myeloma is a malignant hematological neoplasm arising from post–germinal-center B cells
and has a characteristic multistep evolution. An asymptomatic premalignant stage- monoclonal
gammopathy of undetermined clinical significance (MGUS) leads to an intermediate stage - smoldering
multiple myeloma and may progress to active myeloma.
Case report
On 2011, a 64 year old woman with a good performance status and well controlled essential
hypertension was found to have a mild anemia Hb 110g/l) with a ESR of (55mm in 1st hour). The
serum protein electrophoresis (SPE) revealed a monoclonal band of 18.6g/dl. No end organ
involvement was present clinically, biochemically and radiologically. Bone marrow (BM) had <5%
plasma cells (PC). She was followed up as high risk MGUS. Four years later, she presented with
symptomatic anemia (Hb 74g/l) without other myeloma defining events. BM comprised of 46% clonal
PCs (IgG ƙ). She was diagnosed to have active multiple myeloma, stage I risk category according to
International Staging System. Cyclical therapy with Cyclophosphamide, Thalidomide and
Dexamethasone (CTD) was commenced. She achieved a stringent complete remission following five
cycles of CTD. Although she was eligible for an autologous stem cell transplant, neither was this option
available in the country at that time, nor could she afford it in a foreign country. She received monthly
parenteral bisphosphonate for two years. She remained asymptomatic until 3 years from completion
of the last cycle of chemotherapy.
She developed gradually worsening anemia (Nadir of Hb 80g/l). However SPE, urine protein
electrophoresis including immunofixation of serum and urine and serum free light chain (SFLC) ratio
remained normal. Other possible causes for anaemia were ruled out. Shortly she developed renal
impairment and skeletal survey revealed an unstable fracture of neck of left femur and an internal
fixation was performed. Bone biopsy revealed diffuse infiltration by sheets of plasma cells suggestive
of relapsed disease in the form of a non-secretory myeloma.
She was offered four cycles of Bortezomib, Dexamethasone (VD) and two cycles of Bortezomib,
Dexamethasone, Lenalidomide (VRD). The response assessment at the end of six cycles suggested the
complete remission state by presence of < 1% plasma cells in BM. Renal functions gradually improved
with normalizing of creatinine by the end of the fourth cycle. Currently she is asymptomatic and
receiving lenalidomide maintenance for the last three months and going through the tenth year of her
disease.
Discussion
Multiple myeloma is heterogenous - cytogenetically as well as in its clinical behavior in individual
patients. However it is an incurable disease with invariable relapse. Relapse may be identified
biochemically or clinically. The absence of a paraprotein by serum and/or urine immunofixation or a
normal SFLC ratio should not preclude a diagnosis of multiple myeloma or a relapsed disease in non-secretory
form in symptomatic patients.