Haemophagocytic lymphohistiocytosis (HLH) describes a rare, poorly recognised and under-diagnosed immunopathological syndrome whereby there is a highly stimulated yet ineffective multisystem inflammatory response [1]. I present the first case in English literature of Cytomegalovirus (CMV) associated HLH diagnosed by clinical and serological means, and the fourth case of CMV associated haemophagocytic lymphohistiocytosis in an immunocompetent adult, according to HLH-2004 diagnostic guidelines. I include a literature review of CMV associated HLH in adults and raise awareness of checking serum ferritin in patients who present with a sepsis like syndrome. Additionally, this article discusses the merits of HLH-2004 diagnostic work-up without bone marrow biopsy, using clinical and serological means only. I support the reclassification of HLH alongside the other hyperinflammatory syndromes of SIRS, sepsis, septic shock, and MODS to improve understanding and recognition.

/pdf/cytomegalovirus-associated-haemophagocytic-4fmhmpgb3w.pdf

Cytomegalovirus associated haemophagocytic lymphohistiocytosis in the immunocompetent adult managed according to HLH-2004 diagnostic using clinical and serological means only.

Summary The goal of this study was to observe the therapeutic efficacy of cyclophosphamide, vincristine and prednisone combined chemotherapy (COP) in treating adult haemophagocytic lymphohistiocytosis (HLH). Fifteen cases diagnosed with HLH were enrolled in our study. Each of them was treated with the COP regimen. Two of the fifteen patients had autoimmune disease-associated HLH (13.3%), 2/15 had lymphoma-associated HLH (13.3%), 7/15 had infection-associated HLH (IAHLH) (46.7%), and the remaining 4/15 developed the disease in the absence of apparent underlying disease (26.7%). A complete response (CR) was achieved in 7/15 patients (46.7%), while a partial response (PR) was achieved in 5/15 patients (33.3%). With a mean follow-up of 72.5 weeks, the one-year overall survival was 66.7%. HLH of varying aetiology exhibited differing sensitivity and response to the COP regimen. In combination with aetiological and supportive treatment, the COP regimen as a mild and cost-effective chemotherapy is especially favourable for IAHLH, autoimmune disease-associated HLH, and disease severity precluding intensive treatment.

Treatment of hemophagocytic lymphohistiocytosis with cyclophosphamide, vincristine, and prednisone.

La actividad citotóxica de las células natural killer como herramienta diagnóstica en pacientes pediátricos críticos con sospecha de síndrome hemofagocítico

Hemophagocytic lymphohistiocytosis is a life-threatening disease heralded by fever, cytopenia, hepatosplenomegaly, and multisystem organ failure. Its association with genetic mutations, infections, autoimmune disorders, and malignancies is widely reported.A 3-year-old male Arab Saudi patient with insignificant past medical history and parental consanguinity presented with abdominal distension of moderate severity and persistent fever despite receiving antibiotics. This was accompanied by hepatosplenomegaly and silvery hair. The clinical and biochemical profiles were suggestive of Chédiak-Higashi syndrome with hemophagocytic lymphohistiocytosis. The patient received the hemophagocytic lymphohistiocytosis-2004 chemotherapy protocol and had multiple hospital admissions mainly due to infections and febrile neutropenia. After achieving the initial remission, the patient's disease reactivated and did not respond to reinduction with the hemophagocytic lymphohistiocytosis-2004 protocol. Due to the disease reactivation and intolerance of conventional therapy, the patient commenced emapalumab. The patient was successfully salvaged and underwent an uneventful hematopoietic stem cell transplantation.Novel agents such as emapalumab can be helpful for the management of refractory, recurrent, or progressive disease, while avoiding the toxicities of conventional therapy. Due to a paucity of available data on emapalumab, additional data are needed to establish its role in hemophagocytic lymphohistiocytosis treatment. 

/pdf/successful-use-of-emapalumab-in-refractory-hemophagocytic-btp2e17n.pdf

Successful use of emapalumab in refractory hemophagocytic lymphohistiocytosis in a child with Chédiak–Higashi syndrome: a case report

Background & Aims. The hemophagocytic syndrome is a dangerous hyperinflammatory syndrome usually caused by an infection. It is a result of excessive cell activation in the mononuclear phagocyte system which is manifested itself through cytopenia, systemic inflammatory reaction, liver and spleen impairment. Since the disease is rare and its diagnosing is very complicated, this syndrome has not been studied thoroughly and is overlooked very often. The aim of this work is to describe authors’ experience in dealing with the secondary hemophagocytic syndrome (HPS) and to present a literature review.

http://cyberleninka.ru/article/n/vtorichnyy-gemofagotsitarnyy-sindrom-u-vzroslyh-v-klinicheskoy-praktike-gematologa-obzor-literatury-i-sobstvennye-dannye.pdf

Secondary Hemophagocytic Syndrome in the Adult Patients. Literature Review and Authors' Experience

This study was aimed to detect the level of soluble interleukin-2 receptor (sCD25) and cytotoxic activity of NK lymphocytes in patients with hemophagocytic lymphohistiocytosis (HLH), and to explore their clinical significance in HLH. The enzyme-linked immunosorbent assay was used to detect the sCD25 level in serum of 20 patients with HLH, 15 healthy controls, 20 cases of acute myeloid leukemia and 20 cases of systemic lupus erythematosus. The NK cell cytotoxicity in peripheral blood of patients with HLH and controls were detected by flow cytometry with CD107a antibody labeling and LDH release assay. The results indicated that the level of sCD25 in HLH patients was significantly higher than that in healthy controls and disease groups (P < 0.001). The NK cell cytotoxicity in peripheral blood detected by both methods in patients with HLH were lower than that in healthy controls (P < 0.05), and the results detected by flow cytometry correlated significantly with those by LDH release assay (r = 0.73, P < 0.05). It is concluded that detection of sCD25 levels and NK cell activity in peripheral blood in HLH is of great value. Using flow cytometry following CD107a antibody labeling to measure NK activity is a simple, stability, reproducibility method and can be used for clinical diagnosis of HLH.

[Significance of soluble interleukin-2 receptor and NK cell activity in patients with hemophagocytic lymphohistiocytosis].

This study was aimed to investigate the therapeutic effecacy of HLH-2004 chemotherapy in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). 10 cases of sHLH treated with HLH-2004 regimen at our department were analyzed retrospectively. The results showed that 7 patients had clinical response to HLH-2004 regimen, while other 3 patients had no clinical response. 5 cases did not complete initial therapy of 8 weeks. Out of 5 cases, 4 died in the process of chemotherapy, 1 patient abandoned for serious side effects but finally acquired remission following 4 cycles of CHOP regimen. 5 cases underwent the whole courses of initial therapy. Out of 5 cases, 3 patients acquired remission, and other 2 were not well controlled. Out of the 3 patients who had achieved remission, one died of relapse, and other 2 patients kept remission. Out of the 2 patients who were not well controlled, one patient died, but another patient acquired remission after being discharged. It is concluded that patients with infection-associated hemophagocytic syndrome (IAHS) have high rates of remission after receiving HLH-2004 regimen combining with effective antibiotics. However, patients with HLH secondary to EBV (EBV-HLH) or lymphoma (LAHS) have low rates of remission or are easy to get relapse after remission.

[Clinical analysis of 10 cases of secondary hemophagocytic lymphohistiocytosis treated with HLH-2004 chemotherapy].

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[Significance of soluble interleukin-2 receptor and NK cell activity in patients with hemophagocytic lymphohistiocytosis].

[Clinical analysis of 10 cases of secondary hemophagocytic lymphohistiocytosis treated with HLH-2004 chemotherapy].