CASE SERIES OF RARE PLASMA CELL NEOPLASMS
We report a case series of two patients suffering from plasma cell leukaemia.
CASE 1:
A 60 years old female presented with three months history of bone pains, anorexia and weight loss.
On examination, she had TLC=23x 103/µL, Hb=8.7 g/dL, Platelets=303x 103/µL.
Peripheral blood smear showed increased rouleaux formation. There were 55% abnormal mononuclear cells.
Her ESR showed 120 mm fall in first hour.
Serum urea=190 µmol/L
Serum LDH =491
Radiographic skeletal survey revealed multiple osteolytic lesions in almost all the bones of the body.
Her bone marrow aspiration was done which showed abnormal plasma cells (81%) with binucleate and trinucleate forms.
A monoclonal band of IgG was detected on serum immunoelectrophoresis.
Immunophenotyping showed CD 38 positive plasma cells.
A diagnosis of Plasma cell leukaemia was therefore made.
CASE 2:
A 59 years old male presented with two months history of backache. The pain gradually increased in intensity despite medication (analgesics). He became bed bound after one month. There were no other remarkable symptoms.
On examination he was pale.
His CBC showed TLC=69x 103/µL, Hb=5.3g/dL, Platelets =129x 103/µL
ESR showed 110 mm fall in first hour.
Peripheral blood showed markedly increased rouleaux formation and presence of plasma cells/plasmablasts=56%
Renal function tests were within normal limits.
No osteolytic lesions were detected on radiological skeletal survey. Ultrasound abdomen however showed splenomegaly.
Bone marrow aspirate of the patient showed infiltration by abnormal plasma cells (81%) with binucleate and trinucleate forms.
Immunoelectrophoresis showed
Immunophenotyping showed
A diagnosis of Plasma cell leukaemia was made.
DISCUSSION:
It is reported that the median age of presentation of plasma cell leukaemia is more than 50 years. PCL is commonly seen in light chain (Bence-Jones protein) or IgD myeloma and less frequently in IgG or IgA myeloma. The incidence of osteolytic lesions and bone pains is less whereas extramedullary disease, anaemia, thrombocytopenia, hypercalcemia, increased serum β-2 microglobulin, serum LDH levels and renal failure are more commonly observed in patients with PCL. The disease has poor prognosis with a short survival.
In our patients, case 1 had severe bone pains, osteolytic lesions and IgG myeloma (as shown by immunoelectrophoresis). Plasma cell leukaemia in this case probably evolved from multiple myeloma. Case 2 however fulfilled the features mentioned for primary PCL.
PLASMA CELL LEUKAEMIA:
Plasma cell leukaemia (PCL) is a rare and aggressive disorder characterized by the presence of circulating monoclonal plasma cells more than either 2x103/µL or 20% of peripheral blood lymphocytes. PCL can either arises denovo (primary PCL) or may occur as a secondary transformation of multiple myeloma (secondary PCL). Primary PCL comprises 2-5% of cases of plasma cell tumours. Three cases have been reported from Pakistan1.
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