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Capillaritis as a Skin Manifestation of Hepatosplenic Gamma-Delta T-Cell Lymphoma
- Source :
- Journal of pediatric hematology/oncology. 44(3)
- Publication Year :
- 2021
-
Abstract
- We present the case of a 10-year-old boy who was admitted with 3 months of episodic febrile neutropenia and a new petechial rash. Routine bloods identified neutropenia, thrombocytopenia, and a raised alanine aminotransferase. The dermatology team was consulted in light of the symmetrical petechial eruption of the upper torso. A punch biopsy of the lesion was consistent with early capillaritis. The results showed superficial dermal red blood cell extravasation with mild perivascular lymphohistiocytic inflammatory infiltrate. There was no evidence of an atypical lymphoid infiltrate in the skin biopsy. An initial bone marrow aspirate showed an abnormal mature T-cell population consisting of CD4 and CD8 T cells with gamma-delta positivity. Karyotyping was also done, which demonstrated isochromosome 7q. These findings were consistent with a diagnosis of hepatosplenic T-cell lymphoma (HSTL). The patient underwent fourth-line chemotherapy due to refractory relapsing disease but sadly passed away within 12 months of diagnosis. HSTL is a rare and aggressive subset of peripheral T-cell lymphoma. Prognosis is poor with a median survival of1 year from diagnosis. However, reports suggest improved outcomes if intensive, early, high-dose chemotherapy is used alongside hematopoietic stem cell transplantation. Therefore, there is an impetus to attain early diagnosis for aggressive early treatment and improved patient outcomes. Capillaritis, presenting as asymptomatic nonpalpable purpura, can be a rare presenting feature of HSTL. Dermatologists could play a pivotal role in the early recognition of this rare but aggressive hematological malignancy and promote prompt treatment resulting in better patient outcomes.
- Subjects :
- Male
Pathology
medicine.medical_specialty
business.industry
Splenic Neoplasms
Liver Neoplasms
Receptors, Antigen, T-Cell, gamma-delta
Hematology
Capillaritis
medicine.disease
Lymphoma, T-Cell
Oncology
Hepatosplenic Gamma/Delta T-Cell Lymphoma
Pediatrics, Perinatology and Child Health
medicine
Humans
Neoplasm Recurrence, Local
business
Child
Subjects
Details
- ISSN :
- 15363678
- Volume :
- 44
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Journal of pediatric hematology/oncology
- Accession number :
- edsair.doi.dedup.....754f0a01c6de3308c11ce063048819ef