Isolated cutaneous anaplastic large cell lymphoma progressing to severe systemic disease with myocardial involvement and central nervous system infiltration

Pediatr Blood Cancer. 2008 Apr;50(4):879-81. doi: 10.1002/pbc.21357.

Abstract

Anaplastic large cell lymphoma (ALCL) is a rare tumor comprising around 10-15% of childhood lymphomas. We describe the case of a female who initially presented with localized skin disease associated with an insect bite. However, she subsequently relapsed with widespread systemic ALK-positive ALCL that included lymphoma deposits in the myocardium, a very rare manifestation. Her disease responded well to chemotherapy but she later developed a fatal relapse in the CNS. We also present data on an immune response to ALK, demonstrating a fluctuation in the levels of circulating antibodies to ALK corresponding to the different phases of her illness.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Anaplastic Lymphoma Kinase
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Central Nervous System Neoplasms / drug therapy
  • Central Nervous System Neoplasms / genetics
  • Central Nervous System Neoplasms / pathology*
  • Disease Progression
  • Echocardiography, Transesophageal
  • Fatal Outcome
  • Female
  • Heart Neoplasms / drug therapy
  • Heart Neoplasms / genetics
  • Heart Neoplasms / pathology*
  • Humans
  • Lymphoma, Large-Cell, Anaplastic / genetics
  • Lymphoma, Large-Cell, Anaplastic / pathology*
  • Lymphoma, Large-Cell, Anaplastic / therapy
  • Myocardium
  • Oncogene Proteins, Fusion / biosynthesis
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / immunology
  • Protein-Tyrosine Kinases / biosynthesis
  • Protein-Tyrosine Kinases / genetics
  • Protein-Tyrosine Kinases / immunology
  • Receptor Protein-Tyrosine Kinases
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / surgery

Substances

  • Oncogene Proteins, Fusion
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Protein-Tyrosine Kinases
  • Receptor Protein-Tyrosine Kinases