open access

Vol 48, No 3 (2017)
Prace oryginalne / Original research articles
Published online: 2017-07-01
Submitted: 2017-07-10
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Elevated serum concentrations of β-2-microglobulin are often found at the time of diagnosis of hemophagocytic lymphohistiocytosis in adults with lymphoid and myeloid malignancies

Egle Sumskiene12, Ewa Pawłowicz13, Cecilia Kämpe Björkvall4, Maciej Machaczka15
DOI: 10.1016/j.achaem.2017.07.001
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Acta Haematol Pol 2017;48(3):195-201.
Affiliations
  1. Hematology Center Karolinska and Department of Medicine at Huddinge, Karolinska Institutet, Karolinska University Hospital Huddinge, Stockholm, Sweden
  2. Department of Surgery, Antakalnis Outpatient Clinic, Vilnius, Lithuania
  3. 3. Department of Biology and Parasitology, Chair of Biology and Medical Microbiology, Medical University of Lodz, Lodz, Poland, 22nd Kopciński Avenue, 90-153 Lodz, Poland
  4. Department of Medicine, Sunderby Regional Hospital of Norrbotten County, Luleå, Sweden
  5. Neurology Clinic with Brain Stroke Sub-Unit, Clinical Hospital No. 2 in Rzeszow, Medical Faculty, University of Rzeszow, Rzeszów, Poland, Lwowska 60, 35-301 Rzeszów, Poland

open access

Vol 48, No 3 (2017)
Prace oryginalne / Original research articles
Published online: 2017-07-01
Submitted: 2017-07-10

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of immune regulation. In patients over 60 years of age, HLH associated with hematological malignancies (hM-HLH) is the most prevalent. β-2-Microglobulin (B2M) plays an important role in antigen presentation and immunological regulation. Elevated B2M levels reflect T-cell activation. Objective: The aim of this study was to determine serum B2M concentrations in adults with hM-HLH and to interpret its significance in the context of overall survival (OS). Patients and methods: Serum B2M concentration was determined in 31 adults aged 22–84 years at the time of hM-HLH diagnosis. Lymphoid malignancy was diagnosed in 22 patients and myeloid malignancy in 9 patients. Results: The serum concentration of B2M was elevated in 100% of the examined patients. Mean and median serum B2M concentrations were 5.3 and 4.2mg/L, respectively (range 2–17mg/L). We have not found any significant differences in terms of the studied serum B2M concentrations between patients with T/NK-cell lymphomas, B-cell lymphomas, and myeloid malignancies. The outcome of HLH was poor in vast majority of patients with the median OS for the entire group of 46 days. Conclusions: Elevated serum B2M level is a frequent finding at the time of hM-HLH diagnosis in adults. It seems to be a useful indicator of HLH for its early detection and evaluation afterward, as well as for immediate therapeutic intervention. Further prospective studies answering the question whether serum B2M can be used as a prognostic factor in hM-HLH would be of interest.

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of immune regulation. In patients over 60 years of age, HLH associated with hematological malignancies (hM-HLH) is the most prevalent. β-2-Microglobulin (B2M) plays an important role in antigen presentation and immunological regulation. Elevated B2M levels reflect T-cell activation. Objective: The aim of this study was to determine serum B2M concentrations in adults with hM-HLH and to interpret its significance in the context of overall survival (OS). Patients and methods: Serum B2M concentration was determined in 31 adults aged 22–84 years at the time of hM-HLH diagnosis. Lymphoid malignancy was diagnosed in 22 patients and myeloid malignancy in 9 patients. Results: The serum concentration of B2M was elevated in 100% of the examined patients. Mean and median serum B2M concentrations were 5.3 and 4.2mg/L, respectively (range 2–17mg/L). We have not found any significant differences in terms of the studied serum B2M concentrations between patients with T/NK-cell lymphomas, B-cell lymphomas, and myeloid malignancies. The outcome of HLH was poor in vast majority of patients with the median OS for the entire group of 46 days. Conclusions: Elevated serum B2M level is a frequent finding at the time of hM-HLH diagnosis in adults. It seems to be a useful indicator of HLH for its early detection and evaluation afterward, as well as for immediate therapeutic intervention. Further prospective studies answering the question whether serum B2M can be used as a prognostic factor in hM-HLH would be of interest.

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Keywords

Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; β-2-Microglobulin; Hematological malignancy; Survival

About this article
Title

Elevated serum concentrations of β-2-microglobulin are often found at the time of diagnosis of hemophagocytic lymphohistiocytosis in adults with lymphoid and myeloid malignancies

Journal

Acta Haematologica Polonica

Issue

Vol 48, No 3 (2017)

Pages

195-201

Published online

2017-07-01

DOI

10.1016/j.achaem.2017.07.001

Bibliographic record

Acta Haematol Pol 2017;48(3):195-201.

Keywords

Hemophagocytic lymphohistiocytosis
Hemophagocytic syndrome
β-2-Microglobulin
Hematological malignancy
Survival

Authors

Egle Sumskiene
Ewa Pawłowicz
Cecilia Kämpe Björkvall
Maciej Machaczka

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