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Hemophagocytic Lymphohistiocytosis Secondary to Peripheral T Cell Lymphoma with Rapid Onset and Fatal Progression in a Young Patient: A Case Report and Review of the Literature.
Paluszkiewicz, Patrycja; Martuszewski, Adrian; Majcherek, Maciej; Kucharska, Marta; Bogucka-Fedorczuk, Aleksandra; Wróbel, Tomasz; Czyz, Anna.
Afiliação
  • Paluszkiewicz P; Department of Emergency Medical Service, Wroclaw Medical University, Wroclaw, Poland.
  • Martuszewski A; Department of Hygiene, Wroclaw Medical University, Wroclaw, Poland.
  • Majcherek M; Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
  • Kucharska M; Clinic of Infectious Diseases and Hepatology, Wroclaw Medical University, Wroclaw, Poland.
  • Bogucka-Fedorczuk A; Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
  • Wróbel T; Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
  • Czyz A; Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.
Am J Case Rep ; 22: e932765, 2021 Sep 30.
Article em En | MEDLINE | ID: mdl-34588412
BACKGROUND Constant stimulation of lymphocytes and histiocytes can result in hemophagocytic lymphohistiocytosis (HLH), which can be primary or secondary (sHLH). The main causes of sHLH are infections and hematological malignancies, especially non-Hodgkin lymphoma. Despite new insights into the pathogenesis of HLH, the diagnosis and treatment of this immune disorder remain a great challenge. CASE REPORT We present a case of a young adult without comorbidities whose clinical course was nonspecific for several months and resulted in late diagnosis of HLH secondary to peripheral T cell lymphoma (PTCL). The etiological factor of recurring fever, hepatosplenomegaly, and deteriorating condition was unidentified for a long time before fatal sHLH was finally diagnosed. The patient was treated according to the HLH-2004 protocol; however, he did not achieve any response. Unfortunately, due to nonspecific symptoms, lack of lymphadenopathy for a long time, and negative positron emission tomography results, the diagnosis of PTCL was established only after the patient's death. CONCLUSIONS It should be emphasized that early diagnosis is crucial for better prognosis of patients with sHLH. Bone marrow biopsy is worth considering in patients with prolonged fever of unknown origin, hyperferritinemia, splenomegaly, and unexplained cytopenia of 2 or more lineages. Despite the existence of diagnostic and therapeutic protocols available in the literature, the prompt diagnosis and treatment of HLH remains a great challenge. More precise and specific diagnostic tools for HLH are needed.
Assuntos

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Linfoma de Células T Periférico / Linfo-Histiocitose Hemofagocítica Tipo de estudo: Diagnostic_studies / Etiology_studies / Guideline / Prognostic_studies / Screening_studies Limite: Adult / Humans / Male Idioma: En Ano de publicação: 2021 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Linfoma de Células T Periférico / Linfo-Histiocitose Hemofagocítica Tipo de estudo: Diagnostic_studies / Etiology_studies / Guideline / Prognostic_studies / Screening_studies Limite: Adult / Humans / Male Idioma: En Ano de publicação: 2021 Tipo de documento: Article