Introduction:
Mantle cell lymphoma (MCL) is characterized by CD5+ and cyclin D1+ with t(11;14). CD5- is seen in 5% of cases. Two rare cases have been retrieved for hospital record.
Case History:
Case 1: An eighty year old male presented with lymphadenopathy. Peripheral smear & bone marrow (BM) aspirate showed 70% abnormal lymphoid cells. Flowcytometer showed cells, positive for CD 19, CD 20, CD22, CD45 & CD 38 and negative for CD5, CD 3, CD23 & CD 10 with lambda light chain restriction. Serum creatinine was 2.5 mg/ dl. Lymph node biopsy showed lymphoid cells positive for CD20, BCL2, Cyclin D1 & negative for CD3, CD5, CD23, CD10, bcl6 & Annexin A1. FISH showed IgH/ CCND1 translocation [t (11:14)]. CD5 negative MCL was considered. Renal impairment contraindicated BR regime. Management with R-mini CHOP followed by Rituximab maintainence was given. After 3 months inguinal mass was noticed, managed with Radiotherapy. PETCT still showed lymphadenopathy at age 86. Now RCVP + Weekly Bortezumab were advised. Symptomatic improvement was seen.
Case 2: A 55year old female presented with painful swelling in neck & enlarged tonsils. Bone Marrow (BM) aspirate and biopsy showed no lymphoma infiltration. PET-CT showed generalized lymphadenopathy . NHL was suggested.Lymph node biopsy showed lymphoid cell infiltration, positive for CD20, CD5, BCL-2, Cyclin D and negative for CD3, CD10, BCL6, MUM1, CD23. Proliferation marker for Ki 67 was 50%. MCL stage 4 was advised and managed with RCHOP and Rituximab maintenance. She has been spending treatment free interval of >20 months with MIPI 3 (low risk group).
Conclusion:
In the two cases, management was successful with favorable prognosis. Studies suggest that CD5 - MCL have more favorable prognosis than CD5+ one, which may only be differentiated by immunophenotyping.