Random Skin Biopsy Is a Useful Procedure in the Evaluation of Hemophagocytic Lymphohistiocytosis: A Case Report and Review of Literature.
Am J Dermatopathol
; 44(12): 925-928, 2022 Dec 01.
Article
em En
| MEDLINE
| ID: mdl-36197063
ABSTRACT
ABSTRACT Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome, characterized by aberrant activation of T lymphocytes and macrophages leading to hypercytokinemia. HLH can be familial or a result of various secondary etiologies. We present a case of a 46-year-old woman with a past medical history of multiple sclerosis on rituximab who presented as a transfer from an outside hospital with numerous clinical abnormalities including recurrent episodes of fever of unknown origin for 3 weeks, persistent leukocytosis, hypertriglyceridemia, and steatohepatitis. Given the uncertain nature of her illness, she underwent a random skin biopsy from the abdominal region to exclude hematolymphoid malignancy. Histopathology revealed a brisk histiocytic rich dermal infiltrate accompanied by perivascular lymphocytic infiltrate. The histiocytes were enlarged and positive for muraminadase and CD68 stains exhibiting hemophagocytosis focally. As per the HLH-2004 protocol, our patient met the diagnostic criteria of HLH. Concurrent bone marrow biopsy revealed similar rare hemophagocytosis. Cytogenetics and molecular studies were negative, supporting secondary HLH.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfo-Histiocitose Hemofagocítica
Tipo de estudo:
Clinical_trials
/
Guideline
Limite:
Female
/
Humans
/
Middle aged
Idioma:
En
Revista:
Am J Dermatopathol
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Panamá