# T-large Granular Lymphocytic Leukemia: A Rare Diagnosis in a Young Woman With Fever, Necrotic Skin Lesions and Cytopenias

## TL;DR

A young woman with fever, skin lesions, and blood cell issues was diagnosed with a rare type of leukemia called T-large granular lymphocytic leukemia.

## Contribution

This case report highlights the rare presentation of T-LGLL in a young woman with atypical symptoms and severe neutropenia.

## Key findings

- The patient had severe neutropenia and necrotic skin lesions consistent with ecthyma and vasculitis.
- Bone marrow biopsy and flow cytometry confirmed the diagnosis of T-LGLL with CD8+ T-cell infiltration.
- The patient showed significant clinical improvement without specific treatment due to the indolent nature of the disease.

## Abstract

T-large granular lymphocytic leukemia (T-LGLL) is a rare lymphoproliferative disorder. The diagnosis is established by identifying an abnormally high number of clonal granular T lymphocytes in the peripheral blood and eventually in the bone marrow, in cases with medullary infiltration. The majority of patients present with symptoms related to neutropenia and this condition may be associated with autoimmune diseases in up to a third of cases. The authors describe the case of a 26-year-old patient admitted with subacute high fever and bullous dermatitis with necrotic lesions with central bullae. Analytically, she presented anemia and leukopenia with severe neutropenia of 200 cells/L. Skin lesions were compatible with ecthyma and the skin biopsy revealed aspects compatible with leukocytoclastic vasculitis. The myelogram and bone biopsy revealed hypoplasia of the myeloid line and a pathological T population of CD8+, TIA-1+ and granzyme B+, which were associated with compatible flow cytometry (CD3+, T-cell receptor (TCR) Alpha-Beta+, CD5+, CD2+, with loss of CD7 antigen expression) established the diagnosis of T-LGLL. The patient had a favorable evolution, with cytopenias almost returning to normal after two months. She began follow-up at a Hematology Reference Center, remaining asymptomatic without specific treatment considering the indolent course of the disease.