Hemophagocytic lymphohistiocytosis: literature review and clinical case

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Abstract

Hemophagocytic lymphohistiocytosis is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic.

T cells, the cause of which is most often infection. The article presents a clinical case of hemophagocytic lymphohistiocytosis in a 34-year-old patient with a history of follicular lymphoma, who was treated at the A.F. Tsyba Medical Radiological Research Center. Six courses of R-CHOP were performed for follicular lymphoma with a complete response and further supportive therapy with rituximab. A month after the end of therapy, the patient complained of pain in his right side. According to morpho-immunohistochemical examination, the diagnosis of B-lymphoblastic lymphoma was established. Treatment according to the ALL-2016 protocol at the stage of consolidation III was complicated by a long period of three-stage cytopenia with the addition of sepsis. According to the results of an additional examination and assessment according to risk scales, the diagnosis of hemophagocytic lymphohistiocytosis was confirmed. After a few days of therapy with ruxolitinib, the general somatic status of the patient improved, blood parameters were restored and hematopoiesis was normalized according to myelogram data.

A generalized infectious process can be assumed as the cause of the development of hemophagocytic lymphohistiocytosis in this patient.

About the authors

D. A. Manaenkov

A.F. Tsyba Medical Radiological Research Center – branch of the National Medical Research Radiological Center, Ministry of Health of Russia

Author for correspondence.
Email: manaenkov.00@inbox.ru
ORCID iD: 0009-0007-9269-7405

Daniil Arturovich Manaenkov

4 Koroleva St., Obninsk, 249031

Russian Federation

A. B. Galitsyna

A.F. Tsyba Medical Radiological Research Center – branch of the National Medical Research Radiological Center, Ministry of Health of Russia; Obninsk Institute of Atomic Energy – branch of the National Research Nuclear University “MEPhI”

ORCID iD: 0009-0009-0188-8420

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

Russian Federation

N. A. Falaleeva

A.F. Tsyba Medical Radiological Research Center – branch of the National Medical Research Radiological Center, Ministry of Health of Russia; Obninsk Institute of Atomic Energy – branch of the National Research Nuclear University “MEPhI”

ORCID iD: 0000-0002-0023-4216

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

Russian Federation

V. A. Shuvaev

A.F. Tsyba Medical Radiological Research Center – branch of the National Medical Research Radiological Center, Ministry of Health of Russia; Obninsk Institute of Atomic Energy – branch of the National Research Nuclear University “MEPhI”

ORCID iD: 0000-0003-3536-0770

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

Russian Federation

A. A. Vovchenko

A.F. Tsyba Medical Radiological Research Center – branch of the National Medical Research Radiological Center, Ministry of Health of Russia

ORCID iD: 0009-0009-6911-3322

4 Koroleva St., Obninsk, 249031

Russian Federation

S. A. Ivanov

A.F. Tsyba Medical Radiological Research Center – branch of the National Medical Research Radiological Center, Ministry of Health of Russia; Obninsk Institute of Atomic Energy – branch of the National Research Nuclear University “MEPhI”

ORCID iD: 0000-0001-7689-6032

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

Russian Federation

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