Main Article Content
Introduction: Mycosis fungoides (MF) is the most common type of cutaneous T –cell lymphoma accounting for 50% of all cutaneous lymphomas. Sezary Syndrome (SS) and MF are closely related T –cell neoplasms. They are considered separately on the basis of clinical features and cell of origin. Flowcytometry plays an important role in the diagnosis of MF/SS with a characteristic immunophenotypic expression of a lack of CD 7 as a common feature in all stages of the disease. Our case is a clinically asymptomatic patient with a flowcytometric pattern of Mycosis fungoides/Sezary syndrome. There is no documentation of such a case in the literature.
Case: A 55 - year – old male presented with persistent lymphocytosis. Investigations revealed a Hb of 15.5 g/dl, ESR 03 mm/1st hour, platelet count of 178,000/cu mm, total WBC count of 10,700/cu mm and an absolute lymphocyte count of 7000/cu mm (62%). The serum protein electrophoresis was normal. LDH was 149 IU/L (150-250) IU/L. A chest X-ray showed no pathology.
The patient was followed up for a period of five months with full blood counts (FBC), monospot test, ultrasound scan of abdomen, full body CT scan, LDH level and viral studies. During this period, the lymphocytosis persisted. Serial absolute lymphocyte counts were 6332/cu mm, 4918/cu mm, 5749/cu mm, 6890/cu mm and 7820/cu mm. Viral studies revealed CMV IgG antibody positivity and Hepatitis A (HAV) IgG positivity. Studies for HIV were negative. Monospot test for infectious mononucleosis was negative. Ultrasound scan of abdomen and full body CT scan were normal. Blood picture revealed small to medium sized lymphocytes with scanty cytoplasm. Bone marrow examination revealed a reactive marrow with a normal lymphocyte count of 15-20%. Flowcytometry was performed using peripheral blood and bone marrow samples 5 months apart.
Results: Flowcytometry of peripheral blood and bone marrow revealed that T-lymphocytes percentage was 92.0%and 82.0%, respectively. The immunophenotypic results for both blood and bone marrow revealed almost identical findings on LST (Lymphoid Screening Tube) and TCLPD (T Cell Lymphoproliferative Disorder) panels showing bright positivity of smCD3, CD4, CD2, TCRαβ, CD5 and dim positive CD8. Negative results were CD7, TCRγδ, CD25, CD26 and CD4+/ CD8+ ratio was 3.0/2.3 respectively.
Conclusion: The criteria of International Society of Cutaneous Lymphomas (ISCL) and the European Organization for Research and Treatment of Cancer (EORTC) to stage Mycosis fungoides and Sezary Syndrome were not present in our patient who was asymptomatic but showed a typical immunophenotypic pattern of MF/SS.
Gorczyca W. Flowcytometry in neoplastic hematology morphologic–immune-phenotypic correlation. 2nd Ed. 2010;230-234.
Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: A proposal of the International Society for Cutaneous Lymphoma (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713-1722.
Murphy GF, Schwarting R. Cutaneous lymphomas and leukemias. In Lever’s histopathology of the skin. Lippincot Williams and Wilkins. 9th Ed. 2005;927–978.
Khunger JM, Khunger N, Azad K, Srivastava S. Mycosis fungoides: A case report. Indian Journal of Hematology and Blood Transfusion. 2008;26(1):12–14.
Akinbami AA, Osikomaiya BI, John-Olabode SO. Mycosis fungoides: Case report and literature review. Clinical Medicine Insights: Case Reports. 2014;7:95–98.
Wojdyło MS, Rybak WB, Cegielska A. Atopic dermatitis-like Pre-Sézary Syndrome: Role of immunosuppression. Acta Dermato-Venereologica. 2011;91: 574–577.
Berti E, Tomasini D, Vermeer MH, et al. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior. American Journal of Pathology. 1999;155:483–92.
Whittam LR, Calonje E, Orchard G, et al. CD8-positive juvenile onset mycosis fungoides: An immunohistochemical and genotypic analysis of six cases. Br J Dermatol. 2000;143:1199–204.
Foucar K. Mature T-cell leukemias including T-prolymphocytic leukemia, adult T-cell leukemia/lymphoma, and Sezary syndrome. American Journal of Pathology. 2007;127:496–510.
Jones D, Dang NH, Duvic M, et al. Absence of CD26 expression is a useful marker for diagnosis of T-cell lymphoma in peripheral blood. American Journal of Clinical Pathology. 2001;115:885–92.
Izykowska K, Przybylski GK. Genetic alterations in Sezary Syndrome. Journal Leukaemia and Lymphoma. 2011;52(5): 745-753.
Prochazkova M, Chevret E, Mainhaguiet G, et al. Common chromosomal abnormalities in mycosis fungoides transformation. Genes, Chromosomes and Cancers. 2007;46(9):828-838.