Atifa Shuaib, Aqsa Malik.
Plasma Cell Leukaemia - A rare hematological malignancy.
J Coll Physicians Surg Pak Jan ;12(5):318-9.

Plasma cell leukaemia occurs as a primary disorder except in 2-5% of the cases where it occurs as the terminal event in multiple myeloma. It follows a rapidly downhill course with a poor response to therapy and a quick relapse.

A 40 years old female presented to us with a 3-month history of progressively increasing weakness and weight loss. She also complained of backache and chest pain of 15 days duration, which had increased in severity over the last 7 days to the extent that she was bed-ridden and unable to walk. There was no history of bleeding from any site, fever, blurring of vision or headache. She had been previously treated for tuberculosis two and a half years back. There was no history of blood transfusion. Family history was unremarkable. Her examination revealed marked pallor, right supraclavicular lymphadenopathy (0.5 x 1 cm), hepatosplenomegaly and a thyroid swelling (nodule). Her routine investigations were carried out which revealed a normal chemistry (LFTs, RFTs, electrolytes, RBS), urine examination showed traces of albumin. In Blood CP there was severe anaemia of 3.1 gm/dl, a platelet count of 15 x 10e9/l, TLC of 17.7 x 10e9/l. MCV was raised to 115.8 fl. DLC revealed presence of 42% plasma cells with a few myelocytes and metamyelocytes. There was extensive rouleaux formation with an ESR of only 20 mm. Absolute plasma cell count was 7.4 x 10e9/l. Reticulocyte count was 3.8%. A provisional diagnosis of plasma cell leukaemia was made on the basis of peripheral blood film. Considering the clinical and peripheral blood picture following further investigations were carried out: 1. Serum protein electrophoresis was normal. 2. Urine for Bence Jones protein was negative. 3. Serum calcium was normal. 4. X-ray skull and pelvis revealed multiple lytic lesions. 5. Serum immuno-electrophoresis revealed an IgG-kappa paraprotein. 6. Bone marrow showed a presence of 53% plasma cells some of which were abnormal. On the basis of clinical, peripheral blood and bone marrow findings a final diagnosis of plasma cell leukaemia was made. Initially the patient was managed conservatively keeping in mind the poor prognosis of the disease and the economic status of the patient. But later on it was mutually decided among the treating physicians and the haematologist that the patient being young should be given a trial of treatment with CHOP However, there was a delay in the start of treatment. Throughout this time the patient remained febrile and unwell. She was administered first course of CHOP after blood transfusion and antibiotics. After a few days of the first dose when the patient was stable she was allowed to go home with the advice to return after 3 weeks. The patient reported back after one week with a high grade fever and severe anaemia. She was maintained on blood transfusion and antibiotic cover. After an initial short-lived response she relapsed and her condition rapidly deteriorated. She could not be revived and expired in a few days.

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