Research output: Contribution to journal › Article › peer-review
Secondary hemophagocytic syndrome in adult patients. Study of 91 patients. / Potapenko, V. G.; Klimovich, A. V.; Pervakova, M. Y.; Lapin, S. V.; Goloshchapov, O. V.; Titov, A. K.; Surkova, E. A.; Pavluchenko, E. S.; Potikhonova, N. A.; Vinogradova, N. V.; Doguzhieva, E. V.; Kachenya, G. V.; Avdoshina, D. D.; Fedunyak, I. P.; Ryabchikova, V. V.; Kulibaba, T. G.; Rysev, A. V.; Karyagina, E. V.; Medvedeva, N. V.
In: Oncogematologiya, Vol. 15, No. 4, 2021, p. 52-64.Research output: Contribution to journal › Article › peer-review
}
TY - JOUR
T1 - Secondary hemophagocytic syndrome in adult patients. Study of 91 patients
AU - Potapenko, V. G.
AU - Klimovich, A. V.
AU - Pervakova, M. Y.
AU - Lapin, S. V.
AU - Goloshchapov, O. V.
AU - Titov, A. K.
AU - Surkova, E. A.
AU - Pavluchenko, E. S.
AU - Potikhonova, N. A.
AU - Vinogradova, N. V.
AU - Doguzhieva, E. V.
AU - Kachenya, G. V.
AU - Avdoshina, D. D.
AU - Fedunyak, I. P.
AU - Ryabchikova, V. V.
AU - Kulibaba, T. G.
AU - Rysev, A. V.
AU - Karyagina, E. V.
AU - Medvedeva, N. V.
N1 - Publisher Copyright: © 2020 ABV-Press Publishing House. All rights reserved.
PY - 2021
Y1 - 2021
N2 - Background. Secondary hemophagocytic lymphohystiocytosis (sHLH) is a hyperinflammatory reaction provoked by some trigger (cancer, autoimmune or infection). The majority of affected patients are at high risk of fatal multiple organ failure without getting immunsupressive treatment. Objective. Clinical and laboratory profile of sHLH patients. Materials and methods. Retrospective study included clinical, instrumental and lab data from the 91 patients followed between June 2009 and June 2019. Diagnosis sHLH had been based on HLH-2004 and H-Score criteria. The analyzed parameters had been fever chart, liver and spleen enlargement, changes in the bone marrow; values levels of glutamic pyruvic transaminase, serum glutamic oxaloacetic transaminase, alkaline phosphatase, bilirubin, triglycerides, total ferritin with percentage of glycosylation. All patients with rheumatic disorders or malignancies had received either immunosuppressive or cytotoxic therapy. Febrile patients received anti-infective treatment according to the local routine protocols. Results. The data from 91 patients (41 male and 50 female) had been analyzed. Median age was 58 (2-90) years. The sHLH trigger-diseases spectrum included leukemia / lymphoma (n = 52), infection diseases (n = 11), autoimmune disorders (n = 5), allogenic bone marrow transplantation (n = 13), unidentified (n = 10). A fever with an unknown origin and refractory to antibacterial treatment had been observed in 87 (96 %) patients. Morphological hemophagocytic evidences in the bone marrow had been found in 83 %. Breath shortening, liver failure, neurologic disturbances, systemic effusions, rash, heart failure had been registered in 83 % patients. Detected splenomegaly presented in 56 %. Laboratory changes, median were as following: Serum glutamic-pyruvic transaminase (alanine aminotransferase, SGPT) - 92 (39.2-1060.8) IU / L; serum glutamic oxaloacetic transaminase (aspartate aminotransferase, SGOT) - 105 (40-4177) IU / L; alkaline phosphatase - 225 (120.9-989) IU / L; bilirubin - 50.5 (22-559) μmol / L; triglycerides - 3.2 (1.95-8.6) mmol / L; total ferritin - 10 000 (597-255 000) ng / mL with glycosylation percentage - 20.45 (0-37.8) %. 71 patients received various of HLH-directed therapy courses. The overall survival rate was 27 %, median follow-up - 540 days. Conclusion. The main clinical and instrumental findings in sHLH are fever, refractory to anti-infective treatment, elevation of transaminases, serum alkaline phosphatase, triglycerides, total ferritine with low glycosylated fraction. Early diagnosing and immunesupression are the main factors of survival.
AB - Background. Secondary hemophagocytic lymphohystiocytosis (sHLH) is a hyperinflammatory reaction provoked by some trigger (cancer, autoimmune or infection). The majority of affected patients are at high risk of fatal multiple organ failure without getting immunsupressive treatment. Objective. Clinical and laboratory profile of sHLH patients. Materials and methods. Retrospective study included clinical, instrumental and lab data from the 91 patients followed between June 2009 and June 2019. Diagnosis sHLH had been based on HLH-2004 and H-Score criteria. The analyzed parameters had been fever chart, liver and spleen enlargement, changes in the bone marrow; values levels of glutamic pyruvic transaminase, serum glutamic oxaloacetic transaminase, alkaline phosphatase, bilirubin, triglycerides, total ferritin with percentage of glycosylation. All patients with rheumatic disorders or malignancies had received either immunosuppressive or cytotoxic therapy. Febrile patients received anti-infective treatment according to the local routine protocols. Results. The data from 91 patients (41 male and 50 female) had been analyzed. Median age was 58 (2-90) years. The sHLH trigger-diseases spectrum included leukemia / lymphoma (n = 52), infection diseases (n = 11), autoimmune disorders (n = 5), allogenic bone marrow transplantation (n = 13), unidentified (n = 10). A fever with an unknown origin and refractory to antibacterial treatment had been observed in 87 (96 %) patients. Morphological hemophagocytic evidences in the bone marrow had been found in 83 %. Breath shortening, liver failure, neurologic disturbances, systemic effusions, rash, heart failure had been registered in 83 % patients. Detected splenomegaly presented in 56 %. Laboratory changes, median were as following: Serum glutamic-pyruvic transaminase (alanine aminotransferase, SGPT) - 92 (39.2-1060.8) IU / L; serum glutamic oxaloacetic transaminase (aspartate aminotransferase, SGOT) - 105 (40-4177) IU / L; alkaline phosphatase - 225 (120.9-989) IU / L; bilirubin - 50.5 (22-559) μmol / L; triglycerides - 3.2 (1.95-8.6) mmol / L; total ferritin - 10 000 (597-255 000) ng / mL with glycosylation percentage - 20.45 (0-37.8) %. 71 patients received various of HLH-directed therapy courses. The overall survival rate was 27 %, median follow-up - 540 days. Conclusion. The main clinical and instrumental findings in sHLH are fever, refractory to anti-infective treatment, elevation of transaminases, serum alkaline phosphatase, triglycerides, total ferritine with low glycosylated fraction. Early diagnosing and immunesupression are the main factors of survival.
KW - Epstein-Barr virus
KW - Etoposide
KW - Ferritin
KW - Glycosylated ferritin
KW - Haemophagocytic syndrome
KW - Inflammation
UR - http://www.scopus.com/inward/record.url?scp=85099064845&partnerID=8YFLogxK
U2 - 10.17650/1818-8346-2020-15-4-52-64
DO - 10.17650/1818-8346-2020-15-4-52-64
M3 - Article
AN - SCOPUS:85099064845
VL - 15
SP - 52
EP - 64
JO - Онкогематология
JF - Онкогематология
SN - 1818-8346
IS - 4
ER -
ID: 87442201