Development Of Hemophagocytic Syndrome Research Articles

A severe acute cholestatic hepatitis due to visceral leishmaniasis in a child using monoclonal antibodies and systemic steroids

Acute cholestatic hepatitis is a rare but potentially severe manifestation of visceral leishmaniasis. There are few described pediatric cases of acute hepatitis due to leishmaniasis, and to our knowledge, this is the first reported case in a pediatric patient with associated monoclonal antibody therapy. The novelty of monoclonal antibodies requires further investigation of their potential involvement in systemic infections. The development of haemophagocytic syndrome in visceral leishmaniasis is very rare and can represent an additional challenge in diagnosis and management.

Clinical case: observation of a child with Chediak-Higashi syndrome

Chediak-Higashi syndrome is a rare immunodeficiency condition with generalized cellular dysfunction, with an autosomal recessive type of inheritance. Mostly young children suffer, death caused by infections or malignant neoplasms often occurs before they reach the age of 10 years. We describe a case of infectious mononucleosis caused by Epstein-Barr virus in a 3-year—old boy against the background of a rare hereditary Chediak-Higashi syndrome with the development of hemophagocytic syndrome.

Compromised liver function in patients with a new coronavirus infection COVID-19

Clinicians have noted an increase in the level of hepatic transaminase in patients with the new coronavirus infection COVID-19 a number of times, and an unambiguous answer to the question of what caused this change in laboratory indices has not been received yet. The course of COVID-19 is often complicated by the development of haemophagocytic syndrome, and preventive anti-inflammatory therapy is required for its treatment, which is represented by biologic drugs (interleukin-6 inhibitors or JAK-kinase blockers) and glucocorticosteroids. It has been noted that the level of ALT and AST increases against the background of biological therapy.The objective of the present studywas to analyze the possible effect of biological therapy on the functional state of the liver.Material and methods. The study randomly included 38 patients diagnosed with ICD-10 U07.1 (18 women and 20 men). Inclusion criteria were control of aspartate aminotransferase (AST), alanine aminotransferase (ALT), bilirubin at least three times during the period of hospitalization, as well as an increase in these indicators above the reference values. This study was carried out while prescribing preventive anti-inflammatory therapy.Results. The median (Me ± SD) age of the patients was 57 ± 14.67. Mann–Whitney U-test showed no significant difference in the activity of liver enzymes and total bilirubin upon admission to the infectious diseases hospital (p> 0.05). A statistically significant difference in indicators evaluation at admission and at discharge was determined using the Wilcoxon test for ALT levels in the group receiving biologic drugs (p = 0.004). In other cases, there was no statistically significant difference (p > 0.05). When analyzing the level of enzymes for the entire period of hospitalization with the use of Friedman criterion, the level of changes in hepatic transaminases in the group with the use of biologic drugs turned out to be equal for ALT p = 0.001. When assessing changes over the entire period of hospitalization by the de Ritis coefficient, the p-value was also significant (0.001) in this group.Conclusion. This study shows the necessity to control the level of hepatic transaminase, especially when prescribing biological therapy for haemophagocytic syndrome.

Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy. To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge. A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016. The female-to-male ratio was 1.7. The median age at diagnosis was 46.5years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3+, CD4-, CD8+, CD56-, TIA1 cytotoxic granule associated RNA binding protein 1-positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14months. The 5-year disease-specific survival rate was 85.7%. This was a retrospective study. SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.

Cord blood transplantation in Japan.

Cord blood transplantation (CBT) has increasingly been used in Japan and the annual number of CBT now exceeds 1,200. The cumulative number of CBT reached 12,853 in 2015, accounting for almost 1/3 of total CBT performed worldwide. It is true that smaller body size and lower costs, as compared to western countries, have been advantages for Japanese people in using CB as graft alternative. In addition, several novel findings regarding serious issues following CBT have been obtained, which further enhanced the use of CB. First, several mechanisms of engraftment failure following CBT other than cell dose have been reported, such as the presence of donor-specific anti-HLA antibodies or the development of hemophagocytic syndrome. Second, unique profiles of infectious complications following CBT have been reported, such as higher incidences of early bacterial infections and HHV-6 encephalitis, as compared to those following bone marrow (BM)/peripheral blood (PB) transplants. Third, the incidence of disease relapse was comparable to those following BM/PB transplants. Novel pre-transplant conditioning regimens using intravenous busulfan have been investigated with promising results being obtained to date. A recent analysis of Japanese transplant registry data revealed similar survival following CBT to HLA-matched unrelated BM/PB transplants.

Serum β2-microglobulin level is a useful indicator of disease activity and hemophagocytic syndrome complication in systemic lupus erythematosus and adult-onset Still’s disease

This study demonstrates whether serum β2-microglobulin (β2-MG) level can be an indicator of the status of systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD), and development of hemophagocytic syndrome (HPS) complication. Serum β2-MG level was compared between the active and inactive statuses of SLE and AOSD in hospitalized patients. Active status was defined as a state for which a therapy was introduced. Serum β2-MG level was also compared between patients with and without HPS complication. HPS was diagnosed on the basis of clinical and pathological findings. Laboratory markers of HPS including peripheral blood cell counts and levels of serum lactate dehydrogenase (LDH), serum ferritin, plasma fibrin/fibrinogen degradation product (FDP), and plasma D-dimer were examined to determine their correlations with serum β2-MG level. Sixteen SLE and seven AOSD patients (all females, aged 39.0 ± 16.4) were included. The serum β2-MG level was high in the active status of underlying diseases and decreased significantly after the therapy (3.5 ± 1.4 vs. 2.1 ± 0.8 mg/L, p < 0.001). Among patients with active status, the β2-MG level was higher in patients with HPS (two with SLE and three with AOSD) than in patients without HPS (4.9 ± 1.8 vs. 3.3 ± 1.4 mg/L, p < 0.05). Serum β2-MG level significantly correlated with the levels of serum LDH (r(s) = 0.42, p < 0.05), plasma FDP (r(s) = 0.58, p < 0.05), and plasma D-dimer (r(s) = 0.77, p < 0.01). Serum β2-MG level would be a useful indicator of disease activity and development of HPS complication in patients with SLE and AOSD.

T-cell Lymphoma of CD3+CD4+CD56+Granular Lymphocytes with Hemophagocytic Syndrome

A case of T-cell lymphoma of granular lymphocyte (GL) morphology and CD3+CD4+CD56+ phenotype is reported. The lymphoma originated in the subcutaneous tisse of the right thigh and subsequently infiltrated into various organs including the lymph nodes, peripheral blood, bone marrow, skin, lungs, liver, spleen, and peritoneal cavity, and the patient finally developed a hemophagocytic syndrome. The lymphoma cells were positive for CD2, CD3, CD4, and CD56, and negative for CD8, CD 16, CD20, and CD57. An identical clonal rearrangement of the T-cell receptor β chain genes was detected in cells from a lymph node, peripheral blood, and ascites fluid, indicating the monoclonal proliferation of T cells in such organs. This is a T-cell GL lymphoma, expressing the unusual phenotype of CD3+CD4+CD8-CD16−CD56+, which has not been previously reported. The development of hemophagocytic syndrome has also to our knowledge not previously been reported in T-cell GL lymphoma.