Authors: Marie Claudine Francesca B. Perlas, MD, Johannes F. Dayrit, MD, FPDS, Maria Teresita G. Gabriel, MD, FPDS, Cara Lynn Marie N. Chia, MD
Background: Sezary syndrome is an extremely rare leukemic cutaneous T cell lymphoma, with annual incidence of 0.3 to 1 per 100,000, characterized by triad of erythroderma, lymphadenopathy, and circulating Sézary cells in the peripheral blood.
Case Presentation: We present a case of a 64 year-old Filipino female with a 4- year history of recurrent generalized erythematous scaly patches and plaques, diagnosed as mycosis fungoides. Work-up revealed elevated LDH and peripheral blood smear showing atypical mononuclear cells with blast-like features. Blood flow cytometry revealed markedly increased total lymphocyte population and markedly increased Thelper population. CD4/CD8 ratio was elevated at 41. Skin punch biopsy revealed epidermotropism of medium-sized to large lymphocytes, with convoluted nuclei, perinuclear halo and a dermal lichenoid inflammatory infiltrate of atypical lymphocytes which were positive for the immunohistochemical marker CD3. Bone marrow flow cytometry showed bright expression of CD2, CD3, CD4, CD5, CD45 consistent with a mature T-cell neoplasm. Bone marrow biopsy revealed increased number of small to medium lymphocytes with convoluted, irregularly-shaped nuclei, conspicuous nucleoli and condensed chromatin pattern. A diagnosis of Sezary syndrome, stage IVA1 was given and she was co-managed with an oncologist for chemotherapy.
Conclusion: Sézary Syndrome is a rare and aggressive type of cutaneous T-cell lymphoma. This is the first documented case of Sezary syndrome at our institution from a total of 236 mycosis fungoides cases seen from 1998 to 2018. Prognosis is usually poor, with overall median survival of less than 3 years. Early diagnosis and appropriate treatment may be life saving.
Sezary syndrome, mycosis fungoides, prognosis, blood flow cytometry
Authors: Katrina Carmela M. Belen, MD, DPDS, Jasmin J.Jamora, MD, FPDS , Ma. Cecilia P. Ingente MD, DPDSAbstractBackground: Dermoscopy, a non-invasive diagnostic tool, has been proven to improve the diagnostic accuracy of vascular tumors since it can aid in...
A randomized, double-blind, comparative study on the safety and efficacy of virgin coconut (Cocos nucifera l.) oil against 1% hydrocortisone lotion as an anti-inflammatory and antipruritic preparation for mosquito reactions
Authors: Uy, Veronica S, MD; Gracia B. Teodosio, MD, FPDS; Ma. Teresita G. Gabriel, MD, FPDS; Mary Catherine T. Galang, MD; Mohammad Yoga A. Waskito, MD; Johannes F. Dayrit, MD, FPDS Abstract Background: Virgin coconut oil (VCO) has been reported...
Primary Cutaneous Anaplastic Large Cell Lymphoma (PC-ALCL) is a rare Non-Hodgkin lymphoma (NHL) representing approximately 9% of all cutaneous lymphomas.3 It usually manifests as a slow-growing, solitary tumor which has a tendency to regress spontaneously. However, metastasis is reported in 5-10% of cases.