ALK-Positive Histiocytosis with Peripheral Blood Histiocytes: A Case Report

Acta Haematol. 2021;144(2):218-221. doi: 10.1159/000508524. Epub 2020 Jul 28.

Abstract

Histiocytoses are a diverse group of rare, clinically heterogeneous disorders characterised by tissue infiltration of histiocytes, which may result in organ dysfunction and failure. Over 100 different subtypes of histiocytoses have been recognised, including rare cases of ALK-positive histiocytosis. We report a case of histiocytosis in a neonate who presented with refractory thrombocytopenia, anaemia, and intermittent neutropenia. Histiocytes were present in both peripheral blood smears and bone marrow; ALK positivity was demonstrated by immunohistochemistry. Given the scarce reports of this condition, the variable organ involvement, and the different approaches to management in the cases described, we seek to expand the literature by providing a report of our patient whose condition improved without chemotherapy. The presence of histiocytes in peripheral blood smears of patients with this condition has not previously been reported, and it underscores the importance of routine careful evaluation of blood smears.

Keywords: Haematopathology; Histiocytosis; Laboratory haematology; Paediatric haematology.

Publication types

  • Case Reports

MeSH terms

  • Anaplastic Lymphoma Kinase / metabolism*
  • Bone Marrow / pathology
  • C-Reactive Protein / analysis
  • Fluconazole / therapeutic use
  • Histiocytes / metabolism*
  • Histiocytes / pathology
  • Histiocytosis / diagnosis*
  • Histiocytosis / metabolism
  • Humans
  • Infant, Newborn
  • Lung / pathology
  • Steroids / therapeutic use
  • Sulfamethoxazole / therapeutic use

Substances

  • Steroids
  • Fluconazole
  • C-Reactive Protein
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Sulfamethoxazole