Neurocutaneous syndromes and acute lymphoid leukemia: a case report

Authors

Keywords:

neurocutaneous syndromes, tuberous sclerosis, acute lymphoid leukemia.

Abstract

Introduction: Neurocutaneous syndromes comprise a heterogeneous set of hereditary disorders mainly affecting the skin and the central nervous system. Among the conditions included are neurofibromatosis, tuberous sclerosis and von Hippel-Lindau disease, characterized by dysplasia in various tissues and the formation of tumors in various organs. Increased relative risk has also been described for acute lymphoblastic leukemia, chronic myelomonocytic leukemia and non-Hodgkin lymphoma.

Objective: Describe the case of a patient diagnosed with neurocutaneous syndrome (tuberous sclerosis) who developed acute lymphoid leukemia at age six.

Clinical case: A case is presented of a female six-year-old patient with a history of neurocutaneous syndrome, tuberous sclerosis type, diagnosed at age two. The patient started with febrile syndrome, cervical adenopathies and hepatosplenomegaly. The blood count revealed anemia, severe thrombocytopenia and leukocytosis with the presence of blasts, whereas the medullogram showed 90% infiltration by lymphoid blasts, leading to the diagnosis of acute lymphoid leukemia.

Conclusions: Coexistence of neurocutaneous syndromes and acute lymphoid leukemia is not frequent. A case is described of a patient with both conditions who died despite the treatment indicated.

 

Author Biography

Yamelis Babys Silva Cruz, Instituto de Hematologìa e Inmunología

Especialista de I Grado en Medicina General Integral y en Hematología Miembro de la Sociedad Cubana de HEmatología

Published

2021-04-06

How to Cite

1.
Silva Cruz YB, Gil Agramonte M, Chaviano De Leon M, Menéndez Veitía A, Machin García S, Valdés Cabrera F. Neurocutaneous syndromes and acute lymphoid leukemia: a case report. Rev Cubana Hematol Inmunol Hemoter [Internet]. 2021 Apr. 6 [cited 2024 Dec. 26];37(1). Available from: https://revhematologia.sld.cu/index.php/hih/article/view/1156

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Section

Presentaciones de casos

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