Abstract
INTRODUCTION Plasma cell leukemia (PCL) is a rare disorder, characterized by circulating clonal plasma cell.
It accounts for less than 1% of all plasma cell dyscrasias and has a fatal prognosis.
It can be primary or secondary, when there was a previously diagnosed plasma cell dyscrasia.
The median survival is 7–12 moths in the first case and 2 moths in the second.
CASE We present a 54 years old man, diagnosed in November 2003, with multiple myeloma IgA kappa, Bence Jones +, that presented weight loss, retinal hemorrhages, respiratory distress, hepatomegaly, splenomegaly, osteolytic lesions, the cariotype showed hyperploid, chromosome 13 monosomy, translocations t (1,12) and t (4,14).
He was treated upfront with tree cycles of a drug’s combination with melphalan, carmustine, vincristine and dexametasone with no response, therefore, was changed to cyclophosphamide, adriamycin, vincristine, methotrexate and citarabine.
After two cycles, the patient got complete remission.
The patient had a twin brother, and we decided, to do a double transplantation to consolidate the response. The first transplantation was condicionated with carmustine, etoposide, citarabine and melphalan (BEAM), and the second with cyclophosphamide and total body irradiation; the patient remained in complete remission.
Eight months after second transplant was admitted to the hospital with disorientation, bradypsiquia, headache, and sensoriomotor loss of lower extremity.
Laboratory examination showed differential count of leukocytes, haemoglobin and platelets were normal, LDH increase, absence of monoclonal gammophaty in blood and urine by immunofixation, and the brain’s computerized tomography showed multiple intraparenchymatous lesions, with peripheral edema, in both cerebral hemispheres, confirmed by magnetic nuclear resonance, all of that suggested a neoplastic disease.
These lesions were biopsied with the result of multiple myeloma lambda.
The patient died one day after biopsy because intracranial hypertension.
CONCLUSION Plasma cell disease has poor prognosis, and transplantation could be a good option for some patients, but in our case we only achieve to extend life a few months.
Disclosure: No relevant conflicts of interest to declare.
Author notes
Corresponding author
