Plasmapheresis as a Therapeutic Option for Castleman’s Disease [CD].

Castleman’s Disease (CD) is a distinct pathologic entity with clinical heterogeneity. with hyperimmune or immune dysregulation, There is no known standard treatment. A variety of therapies have been tried including Surgery, Radiation, steroids, single agent chemotherapy, multi agent chemotherapy, Rituximab and monoclonal antibody to IL-6. Plasmaphersis which was successful in this patient has not been tried before in CD.

Case report

31 year old female with no significant past medical illness admitted with 10 days history of fever, abdominal pains. CT scan of chest and abdomen showed small mediastinal and left axillary lymphadenopathy.

Physical examination revealed a palpable 2 cm rubbery mobile non tender left axillary node and mild tenderness over right upper quadrant, otherwise unremarkable

Laboratory examination on admission showed BUN 17 mg/dL, Creatinine 1.4 mg/dL, Potassium 5.5 meq/L, Calcium 8.4 mg/dL, Total Protein 6.6 g/dL, Albumin 2.9 g/dL, Bilirubin 1 mg/dL, Alkaline phosphatase 427 U/L, AST 27 U/L, ALT 26 U/L, GGT 288 U/L. WBCs 12,400, with 70% Neutrophils, 7%Bands, 10%Lymphocytes, 11% Monocytes and 2%. Eosinophils, HGB 12 g/dL, Platelets 630,000. Peripheral smear was normal. ESR 65 mm/hr. ANA-negative. Serum Complement C4 22 mg/dL and C3 116 mg/dL. Monospot test- negative. Toxo titer was negative. PT 15.5” and PTT 38”. Serum Protein Electrophoresis and seum immunofixation were normal. Urine Bence Jones Protein was negative. Serum Cryoglobulin was negative. Urinalysis was normal. Hepatitis profile was negative. Hepatobiliary scan was normal.

Hospital Course-

She continued to have fever despite negative cultures. Renal functions worsened over the first few days of admission, BUN and Creatinine increased to 27 mg and 2.3 mg respectively. Left axillary lymph node biopsy showed Hyaline vascular Castleman’s disease like changes. Biopsy of right kidney was done showed Chronic Thrombotic microangiopathy with predominant glomeruler involvement. Bone marrow biopsy and Immunohistochemistry of the aspiration were normal. HHV- 8 antibody was negative. HIV screening was also negative. She was started on Prednisone 1mg/kg/day without clinical or laboratory improvement. BUN increased to 101mg/dL and Creatinine to 3.8mg/dL with generalized anasarca and reduced urine out put. Hgb dropped to 6.7gms/dl, Platelets 27,000 and WBCs 18,700 with neutrophilia. Peripheral smear showed no schistocytes at any time. Since her condition was worsening despite high dose of steroids, patient was started on daily Plasmaphersis with noticeable daily clinical and laboratory improvement. After 26 sessions of Plasmapheresis all the hematological and renal abnormalities improved with clearance of anasarca and normal urine out put. At the time of discharge - CBC showed WBCs 13,000, Hgb 11.1 g/dL, Platelets 123,000. BUN 25mg/dL and Creatinine 0.5 mg/dL.

The repeat CT scan revealed no lymphadenopathy. Two year later she remains in complete remission with normal renal function and normal blood counts.

Conclusion

Plasmaphersis can help in controlling the Hyper immune or immune dysregulation, consequences of Castleman’s disease and may be an option in treating severe systemic manifestations of CD.