An unusual lupus like malar rash pattern with hepatosplenomegaly as a presentation of gamma delta t cell lymphoma
نویسندگان
چکیده
About 12% of all lymphoid tumors worldwide are peripheral t cell lymphomas, which uncommon subtypes non-hodgkin lymphoma. they have a poor prognosis and more common in populations countries asia. T lymphomas with gamma delta receptors expressed them very rare (less than 1% neoplasms) extremely aggressive; originate from cells; small subset cells direct antigen recognition capability acting at the interface between innate adaptive immunity .
 Lymphoid tissue, skin, gastrointestinal tract, red pulp spleen where lymphocytes tend to cocentrate, representing only 1-5% total circulating lymphocytes, but accounting for up 30% entire population.
 In contrast alpha beta cells, develop cd4/cd8 thymic precursors bone marrow usually lack major histocompatibility complex restriction.
 These conduct as cytotoxic similar natural killer their activity early effectors innate, non-specific immune response capable receptor rearrangement well phagocytosis.
 Hepatosplenic lymphoma primary cutanous recognized two different entities.
 A characterized extranodal lymphoma, hepatosplenic has disguised onset, secondary intrasinusoidal infiltrationn spleen, liver, marrow, rapidly progressive course, is poorly responsive chemotherapy.
 panniculitis-like clinical picture prominent epidermal involvement can be presentation cutaneous lymphoma.
 this case study , authors will discuss 19 year old male patient present lupus like malar rash hepatosplenomegaly who had been diagnosed
منابع مشابه
A strange lupus-like malar rash with renal involvement: an angioimmunoblastic T-cell lymphoma – A case report
Cutaneous malar rash and kidney involvement has not previously been reported as presenting symptoms of an angioimmunoblastic T-cell lymphoma (AITL). We report a case of a woman with erythematous rash. A PET-CT revealed a lymphadenopathy and splenomegaly. An inguinal lymph node biopsy showed an AITL. There was clinical improvement after prednisone.
متن کاملUnusual Presentation of T-cell non-Hodgkin Lymphoma with Multiple Skin Nodules
Non-Hodgkin lymphoma is very uncommon in infancy and skin as the primary site of involvement in Non-Hodgkin lymphoma is rarely encountered. We describe a 10-month-old infant with T-cell Non-Hodgkin lymphoma who presented with multiple skin nodules as the predominant feature of her disease. The clinical manifestations, treatment strategy and disease outcome are reviewed. Key words: Non-Hodgk...
متن کاملAn Unusual Case of Hepatosplenomegaly with Cytopenia
Autoimmune lymphoproliferative syndrome (ALPS) is a rare, inherited disorder of immune dysregulation secondary to defective lymphocyte apoptosis. This leads to uninhibited proliferation of lymphoid tissue manifesting with lymphadenopathy, hepatosplenomegaly, autoimmune cytopenia, and an increased risk of lymphoid malignancy. We report a 2 year old boy with fever, generalized lymphadenopathy, he...
متن کاملDevelopment of a reticular rash in a febrile woman: An unusual cutaneous presentation of angioimmunoblastic T-cell lymphoma
A ngioimmunoblastic T-cell lymphoma (AITL) is a peripheral T-cell lymphoma with a poor prognosis in which 50% of patients present with a rash. Although AITL’s cutaneous presentation is nonspecific, to our knowledge, a reticular rash is not described in published literature. Herein, we report a patient who presented with a generalized, netlike rash, confirmed to be AITL upon histologic analysis ...
متن کاملAn unusual presentation of adult T-cell leukemia/lymphoma
Adult T-cell Leukemia/Lymphoma (ATL) is a rare disease, related to human T-lymphotropic virus-1 (HTLV-1) and presented mainly in adulthood by generalised lymphadenopathy, hepatosplenomegaly, skin lesions and hypercalcaemia, with rare gastrointestinal and/or oral manifestations. We reported this case to raise awareness and demonstrate the therapeutic challenges of this rare disease. A 49-year-ol...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: International journal of scientific research and management
سال: 2023
ISSN: ['2321-3418']
DOI: https://doi.org/10.18535/ijsrm/v11i01.mp04