Hemophagocytic Syndrome In Children Research Articles

Clinical Crrelation and Prognostic Analysis of ALBI Score in Secondary Hemophagocytic Syndrome in Children

To explore the clinical correlation and prognostic value of the Albumin-Bilirubin (ALBI) score in children with secondary hemophagocytic syndrome(sHLH). A retrospective analysis was conducted on the data of children's sHLH cases clearly diagnosed in the Affiliated Hospital of Zunyi Medical University from January 2012 to March 2023. Survival analysis was conducted according to the ALBI classification. Spearman correlation analysis was conducted between the ALBI score and clinical indicators. The Receiver Operating Characteristic(ROC) curve was used to evaluate the ALBI score, select the best cutoff value, and evaluate the accuracy of prognostic prediction value. Kaplan-Meier method was used to draw the survival curve. Log-rank method was used to compare the differences of survival curve between groups. Cox regression was used for prognostic analysis and restricted cubic spline curves used to calculate the relationship between ALBI scores and the risk of death in children with sHLH. A total of 128 children with sHLH were included in this study, with a median age of 38(13.25, 84) months. There were 70 males (54.69%) and 58 females (45.31%). The survival analysis results of ALBI grading showed that the survival rate of HLH patients with ALBI grade 3 was significantly lower than those with ALBI grades 1 and 2. Spearman correlation analysis results showed that ALBI score was positively correlated with splenomegaly, respiratory failure, disseminated intravascular coagulation(DIC), pulmonary hemorrhage, gastrointestinal hemorrhage, central nervous system involvement, ALT, AST, TG, LDH, PT, APTT, and SF (the correlation coefficients are: r =0.181, 0.362, 0.332, 0.221, 0.351, 0.347, 0.391, 0.563, 0.180, 0.448, 0.483, 0.37, 0.356), and was negatively correlated with HB, PLT, and FIB (the correlation coefficients are: r =-0.321, -0.316, -0.423), but was not significantly correlated with EBV infection, fungal infection, hepatomegaly, and ANC (P ＞0.05). Using the ROC curve, the cutoff value of ALBI was -1.76. Single factor Cox regression analysis results showed that HB< 90 g/L, ALT≥80 U/L, AST≥200 U/L, LDH≥1 000 U/L, PT≥20 s, APTT≥40 s, FIB< 1.5 g/L, ALBI≥-1.76, combined pulmonary hemorrhage, DIC, central nervous system involvement, gastrointestinal bleeding, and not using blood purification may be the prognostic risk factors for children with sHLH (P < 0.05). Multivariate Cox regression results showed that FIB< 1.5 g/L (HR =2.119, 95% CI :1.028-4.368), ALBI≥-1.76 (HR =2.452, 95%CI :1.233-4.875), and central nervous system involvement (HR=4.674, 95%CI :2.486-8.789) were independent risk factors affecting prognosis, while blood purification (HR =0.306, 95%CI :0.153-0.612) was an independent protective factor for prognosis. The application of restricted cubic splines shows that the risk of death increases with the increase of ALBI score. The area under the ROC curve (AUC) of the ALBI score for predicting the risk of 1-week, 2-week, 4-week, and overall mortality were 0.825, 0.807, 0.700, and 0.693, respectively, indicating good predictive performance for early mortality risk. According to subgroup analysis results of clinical manifestations, compared with the ALBI < -1.76 group, ALBI≥-1.76 was associated with age ≤2 years, EBV infection, HLH-1994/2004 treatment, concomitant respiratory failure, and ANC≤1.0×10 9/L, HB< 90 g/L, PLT < 100×109/L, TG≥3.0 mmol/L, LDH≥1 000 U/L, APTT≥40 s, and FIB< 1.5 g/L (P < 0.05). The ALBI score is related to the clinical characteristics and laboratory indicators of sHLH, and can be used as a beneficial indicator for assessing the prognostic risk of sHLH in children. It has good accuracy and clinical application value in predicting the prognosis of sHLH in children.

Clinical features and prognostic factors of severe hemophagocytic syndrome in children

Objective: To explore the clinical characteristics, prognostic risk factors and effective treatment of severe hemophagocytic syndrome (HPS) in children, so as to provide reference for the clinical diagnosis and treatment of the disease. Methods: The clinical data of 83 children with severe HPS admitted in Affiliated Hospital of Zunyi Medical University from January 2014 to April 2021 were collected, and their clinical characteristics, prognosis and prognostic risk factors were analyzed. The children were divided into central nervous system (CNS) dysfunction group and non-CNS dysfunction group according to whether they were accompanied with CNS dysfunction, and were divided into blood purification group and non-blood purification group according to whether they received blood purification, then the survival differences were compared. Results: Among the 83 children, there were 43 males and 40 females, aged[M(Q1,Q3)] 36(15,27)months. A total of 51 children were induced by infection, among which 41 children (80.4%) were infected with EB virus. All the children were accompanied by multiple organ dysfunction (MODS), and dysfunction of the blood system (72.3%), liver (71.1%), respiratory system (53.0%) and CNS (37.3%) were common. By the end of follow-up, 40 cases (48.2%) survived, 38 cases (45.8%) died, and 5 cases (6.0%) were lost to follow-up. CNS dysfunction was a risk factor (HR=3.358, 95%CI: 1.445-7.803, P=0.005) and blood purification was a protective factor (HR=0.362, 95%CI: 0.179-0.730, P=0.005) affecting the prognosis of children. The mortality of CNS dysfunction group was statistically higher than that of non-CNS dysfunction group (74.2% vs 28.8%) (P<0.001); The mortality of blood purification group was statistically lower than that of non-blood purification group (31.0% vs 61.0%) (P=0.010). Conclusions: Severe HPS in children was dangerous and had a poor overall prognosis. CNS dysfunction was a risk factor for death. Blood purification could significantly improve the prognosis and improve the survival rate of children.

Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases

GIRIS ve AMAC: Hemofagositik sendrom (HFS) asiri immun aktivasyona bagli hizla ilerleyen ve hayati tehdit eden bir sendromdur. HFS ile iliskili klinik tablo ve laboratuvar anormalliklerinin erken taninmasi ve tedaviye hemen baslanmasi hayat kurtarici olabilmektedir. Bu calismada enfeksiyon iliskili hemofagositik sendrom (EIHFS) tanisiyla izledigimiz cocuk hastalarda; altta yatan enfeksiyoz etkenlerin, klinik, laboratuvar bulgularin ve tedavi sonuclarinin incelenmesi amaclanmistir.YONTEM ve GERECLER: Aralik 2012 ile Ocak 2016 arasinda EIHFS tanisi konan cocuk hastalarin kayitlari geriye donuk olarak incelendi.BULGULAR: Calismaya yaslari ortanca 6 yas (2-16 yas) olan toplam 15 hasta (8 erkek, 7 kiz) dahil edildi. HFS’yi tetikleyen en sik enfeksiyoz etken olarak Brusella (8 hasta, %53) saptandi. Diger altta yatan enfeksiyoz etkenler; 3 hastada Leishmania, 1 hastada Salmonella Typhi, 1 hastada Mycobacterium tuberculosis, 1 hastada parvovirus B19 ve 1 hastada influenza A (H3N2) idi. Ates, bi/pansitopeni, hiperferritinemi ve serum transaminaz duzeylerinde yukseklik tum hastalarda (%100), splenomegali 12 hastada (%80) mevcuttu. Tum hastalarin kemik iligi aspirasyon incelemesinde hemofagositoz goruldu. Tum hastalara altta yatan enfeksiyonuna yonelik antimikrobiyal tedavi; uzamis ates ve klinik durumunda giderek kotulesme olan 8 hastaya intravenoz immunglobulin (IVIG), 2 hastaya IVIG+steroid tedavileri verildi. Olgularin %93’unde (14 hasta) iyilesme kaydedildi. Influenza A (H3N2) pnomonisi olan sadece 1 hastada (%6) olum goruldu. TARTISMA ve SONUC: Hekimler, enfeksiyon nedeniyle izlenen hastalarda uzamis ates, splenomegali ve sitopeninin varliginda HFS gelisimi konusunda farkindaliga sahip olmalidir.

Diagnostic and prognostic value of CD163 for infection-associated hemophagocytic syndrome in children

Objective To investigate the diagnostic and prognostic value of serum soluble CD163 (sCD163) and the positive rate of membrane-bound CD163 (mCD163) in peripheral blood mononuclear cells (PBMC) in children with infection-associated hemophagocytic syndrome (IAHS). Methods Between July 2012 and June 2016, 26 pediatric patients with IAHS (IAHS group) and 28 pediatric patients with sepsis(sepsis group)admitted to Children′s Hospital Affiliated to Shanghai Jiaotong University were selected, and 20 healthy children were taken as healthy control group.Sandwich enzyme linked immunosorbent assay was used to detect serum sCD163.The population of circulating mCD163 positive monocytes was determined by using flow cytometry.Receiver operating characteristic (ROC) curves were used to evaluate the diagnostic and prognostic values of sCD163 and mCD163 in children with IAHS compared with the diagnostic and prognostic values of plasma ferritin, and so on. Results The serum levels of sCD163 in patients of IAHS group, sepsis group and healthy control group were (1 264±538) mg/L, (862±332) mg/L, (610±316) mg/L, respectively.And the population of mCD163-positive PBMC in patients of IAHS group, sepsis group and healthy control group was (88.3±9.7)%, (68.5±18.3)%, (28.9±5.2)%, respectively.Both serum sCD163 and the population of mCD163-positive PBMC were significantly higher in IAHS group compared with those of sepsis group (t=2.031, P=0.048; t=3.191, P=0.002, respectively). The serum sCD163 and population of mCD163-positive PBMC in sepsis group were higher than controls (t=3.848, P=0.002; t=4.049, P=0.000, respectively). Moreover, the areas under the ROC curve (AUC) for the mCD163, sCD163, were 0.853(P=0.013), 0.762(P=0.004), 0.755(P=0.049), respectively.mCD163 at a cutoff of 83.7% had a high diagnosis sensitivity (81.8%) and specificity (72.4%). The optimal cutoff values of sCD163 and ferritin for predicting IAHS was 888 mg/L (sensitivity 66.7% and specificity 63.3%) and 2 880 μg/L (sensitivity 80.0% and specificity 54.5%). In addition, the serum level of sCD163 and the population of mCD163-positive PBMCs were significantly increased in acute phase and decreased in recovery phase[(1 553±542) mg/L vs. (866±92) mg/L, (91.0±6.4)% vs.(79.0±4.6)%, t=2.450, χ2=3.419, P=0.036, 0.007] in IAHS group.Furthermore, subgroup analysis indicated that the serum level of sCD163 and the population of mCD163-positive PBMCs were significantly higher in dead patients than those in survived patients [(1 748.91±518.17) mg/L vs.(909.69±171.35) mg/L, t=3.070, P=0.011; (93.50±8.42)% vs.(77.30±3.28)%, χ2=3.005, P=0.024, respectively]. Conclusion Serum sCD163 and the population of mCD163-positive PMSCs are specific and validity biomarkers for early diagnosis of IAHS, which also are associated with treatment response assessment and prognostic analysis in IAHS. Key words: Soluble CD163; Membrane-bound CD163; Infection-associated hemophagocytic syndrome; Sepsis; Child

Вторичный гемофагоцитарный синдром у детей

Вторичный гемофагоцитарный синдром у детей

Hemophagocytic Syndrome in Children With Visceral Leishmaniasis.

Hemophagocytic lymphohistiocytosis (HLH) is a serious complication of visceral leishmaniasis (VL). The aim of this study is to describe demographical, clinical and laboratory features of HLH in children with VL. This is a retrospective cohort of children with HLH and VL admitted to a tertiary hospital in Northeast, Brazil, from January 2012 to April 2014. Clinical and laboratory data at admission and during hospital stay were reviewed. Acute kidney injury (AKI) was defined according to the pediatric Risk, Injury, Failure, Loss, End-stage kidney disease criteria. A total 127 VL children were admitted, and 35 children had diagnosis of HLH. Mean age was 4.2 ± 4.3 years, with 62.9% males. Mean hospital stay was 29 ± 12 days. Main signs and symptoms were fever (100%), splenomegaly (94.2%) and hepatomegaly (60%). Laboratory findings showed pancytopenia, albumin 3.03 ± 0.77 g/dL, fibrinogen 236.1 ± 117.2 mg/dL, total calcium 8.2 ± 1.2 mEq/L, lactate dehydrogenase 1804 ± 1019 mg/dL, alkaline phosphatase 1275.4 ± 2160.5 IU/L, total bilirubin 1.9 ± 2.4 mg/dL, direct bilirubin 0.67 ± 1.02 mg/dL, indirect bilirubin 1.2 ± 2.2 mg/dL, aspartate aminotransferase 140.0 ± 145.3 IU/L, alanine aminotransferase 71.4 ± 81.1 IU/L, ferritin 4296.5 ± 8028.8 ng/dL and triglycerides 333 ± 141 mg/dL. AKI was observed in 16 children (45.7%), predominantly mild forms (93.75% "risk"). AKI group presented lower levels of platelets (69,131 ± 40,247 vs. 138,678 ± 127,494/mm, P = 0.035) than non-AKI. No patient required dialysis and there was no death. HLH was not a rare complication of VL. Main symptoms were compatible with both VL and HLH. Main laboratory findings reflected HLH pathophysiology. Mild forms of AKI were a common complication of HLH. Despite the disease severity and complications, mortality was low.

Hemophagocytic syndrome in children and adults.

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a heterogenic syndrome, which leads to an acute, life-threatening inflammatory reaction. HLH occurs both in children and adults, and can be triggered by various inherited as well as acquired factors. Depending on the etiology, HLH can be divided into genetic (i.e., primary) and acquired (i.e., secondary) forms. Among genetic HLH forms, one can distinguish between familial HLH and other genetically conditioned forms of HLH. Acquired HLH can be typically triggered by infections, autoimmune diseases, and malignancies. The most common symptoms of HLH are unremitting fever, splenomegaly, and peripheral blood cytopenia. Some severely ill patients present with central nervous system involvement. Laboratory tests reveal hyperferritinemia (often >10,000 μg/L), increased serum concentration of soluble receptor α for interleukin-2 (>2,400 U/L), hypertriglyceridemia, hypofibrinogenemia, coagulopathy, hyponatremia, hypoproteinemia, and elevated liver transaminases and bilirubin. Prognosis in HLH is very serious. Genetic HLH is always lethal if adequate therapy is not administered. Similarly, severe acquired cases often lead to death without appropriate treatment. Since HLH can be encountered by various specialists in the medical field, basic knowledge of this entity such as diagnostic criteria and treatment should be familiar to all physicians.

Hemophagocytic syndrome in children with acute monoblastic leukemia—another cause of fever of unknown origin

Intensification of antileukemic treatment and progress in supportive management have improved the survival rates of children with acute myeloid leukemia (AML). However, morbidity and early mortality in these patients are still very high, especially in children with acute monoblastic leukemia (AML FAB M5). Inflammatory syndromes complicating the management of these children after application of cytosine arabinoside and due to hyperleukocytosis at initial presentation have been reported. Hemophagocytic lymphohistiocytosis (HLH) has been described as a serious and life-threatening acute complication during treatment of different oncologic entities; however, data on HLH in children with AML FAB M5 are extremely rare. A retrospective study of all children with AML FAB M5 treated at our institution between 1993 and 2013 was performed to describe the clinical characteristics of patients who developed an inflammatory syndrome with HLH during oncologic treatment. Three of 10 children developed an inflammatory syndrome with fever, elevation of C-reactive protein, hyperferritinemia, elevation of soluble interleukin-2, and hemophagocytosis during prolonged aplasia following the first cycle of chemotherapy not responding to broad-spectrum antibiotics. No infectious agents could be identified; the initial symptoms occurred 17, 18, and 28days after diagnosis of AML, respectively. The children immediately responded to dexamethasone; however, the same syndrome was observed again after the second cycle of chemotherapy and, in one patient, also after the third cycle. Treating physicians should be aware of an inflammatory syndrome resembling HLH in children with monoblastic leukemia since this problem might extremely complicate management and supportive care of these children. The co-incidence of monoblastic leukemia with HLH might be explained by cytokines released from the monoblastic leukemic cells themselves.

Clinical analysis of Epstein-Barr virus-associated hemophagocytic syndrome in children

The primary infection of Epstein-Barr virus (EBV) may results in hemophagocytic syndrome, known as EBV-associated hemophagocytic syndrome (EBV-AHS), but the clinical risk factors complicating this fatal disease in children with infectious mononucleosis (IM) are unknown. The aim of this study was to identify clinical features of EBV-AHS and to evaluate the curative effect of HLH-2004 protocol. The clinical and laboratory data of 644 IM children including 27 children developed into EBV-AHS and 43 HPS children associated with other diseases were retrospectively analyzed and logistic regression was used to identify the clinical risk factors complicating EBV-AHS. The results showed as follows: (1) the prevalence of EBV-AHS in IM children was 4.2% (27/644), and the prevalence in group aged younger than 3 years was higher than in other age groups. The incidence age of EBV-AHS was significantly younger than that of other HPS patients; (2) Liver function damage of group aged older than 7 years was much more severe in HPS patients. (3) Compared with other HPS patients, male patients were more common and liver function damage was severe in EBV-AHS patients, especially in the patients aged at 2 years or younger. (4) The fatality rate in the EBV-AHS patients was 37.0% (10/27). (5)After treatment with HLH-2004 protocol, the fatality rate in patients with EBV-AHS decreased from 50.0% to 18.2%, the overall survival (OS) of 3 years significantly increased (P = 0.032). It is concluded that IM is a benign self-limited disease, of which only about 4.2% patients will develop into EBV-AHS. Clinical risk factors identified in this study may be helpful for early diagnosis of IM children with complicated EBV-ASH, the HLH-2004 protocol can obviously improve prognosis of EBV-HPS.

Detection of Th1/Th2 Cytokines Is Useful for the Quick and Early Diagnosis of Hemophagocytic Syndrome in Children.

Introduction and objectives Hemophogocytic syndrome (HPS) is an infrequent disease in children with a high mortality rate. Currently, the diagnosis of HPS largely depends on non-specific clinical manifestations, but the clinical features allowing to meet the criteria of diagnosis of HPS usually do not appear simultaneously which make the early diagnosis very difficult. According to the literature, hypercytokinemia has been noticed for this disease. However, the pattern of hypercytokinemia in HPS has not been proposed. In this study, the quick determination of Th1/Th2 cytokines in the sera from patients with HPS was performed and the patterns of the cytokines were analyzed to seek for the possibility of using the cytokine pattern for the early diagnosis of HPS and its prognostic significance.Patients and Methods A total of 16 children with HPS based on the 2004 HPS criteria from June 2005 ∼ December 2006 were enrolled into this study. Peripheral blood samples were collected at the time of diagnosis, during the induction therapy and at the time of relapse. Sera from 21 healthy children, 17 ALL at remission and 17 with sepsis were used as controls. A cytometric bead assay (CBA) was used to quantitatively determine the Th1/Th2 cytokines including IL-2, IL-4, IL-6, IL-10, TNFα and IFNγ.Results Levels of IL-6 (22.20 (10.60–139.25) pg/ml), IL-10 (195.60(63.85–918.70)pg/ml) and IFNγ (4740.20(1054.72– >5000.00)pg/ml) from patients with HPS were significantly higher than those of normal control (3.35 (2.50–5.40) pg/ml, P < 0.05; 2.90(2.20–4.80)pg/ml, P<0.01; 52.80(37.50–66.60)pg/ml, P<0.01). Although the IFNγ levels in septic patients were also elevated, the levels (4740.20(1054.72- >5000.00)pg/ml) of IFNγ in HPS patients were much higher than those (52.80(37.50∼66.60)pg/ml, P<0.01) in septic patients. Another important finding was that the levels of IL-6 in HPS (22.20(10.60∼139.25)pg/ml) were significantly less elevated than those in septic patients (526.55(194.80∼979.00)pg/ml, P<0.01). After effective therapy with HLH2004 protocol, the IL-6 (3.30(2.30∼6.80)pg/ml, IL-10(6.60(3.10∼23.20)pg/ml and IFNγ (65.45(27.30∼144.10)pg/ml) levels from patients with HPS sensitive to chemotherapy were significantly decreased as compared to those (22.20(10.60∼139.25)pg/ml, P=0.005; 195.60(63.85∼918.70)pg/ml, P=0.004; 4740.20(1054.72∼ >5000.00)pg/ml, P=0.002) of prior to treatment. While those in patients insensitive to chemotherapy were not significantly reduced (P > 0.05). No significant differences in the IL-2, IL-4 and TNFα levels among patients at acute phase, remission stage and the healthy controls were observed. Univariate analysis showed that the initial IL-10 level >100pg/ml and IFNγ concentration > 4000pg/ml were both unfavorable prognostic factors for HPS (IFNγ, P =0.034; IL-10, P=0.034). Positive correlations between the levels of IFNγ and IL-10(r=0.893, P<0.001), IFNγ and IL-6(r=0.791, P<0.001), IL-10 and IL-6(r=0.676, P<0.001) were found. All of the three parameters stayed at a high level in acute phase and returned to normal when achieving remission.Conclusion s The quick determination of Th1/Th2 cytokines is a useful approach for the early and quick diagnosis in HPS patients based on the pattern of significant elevations of IFNγ and IL-10 with slight elevation of IL-6 and it can be used for the monitoring of the progress and the prognosis of the disease.

Enterovirus-associated hemophagocytic syndrome in children with malignancy: report of three cases and review of the literature

Enteroviruses can cause severe manifestations in children with malignancy. Infection-associated hemophagocytic syndrome (IAHS) due to enterovirus is a rare entity in children. Patients with malignancy and IAHS due to enterovirus were retrospectively evaluated at the University of Athens' Hematology-Oncology pediatric unit within a 6-year period (2000-2006). IAHS occurred in three cases among 56 patients with documented enteroviral infection. The diagnosis of IAHS was confirmed by bone marrow aspiration and biopsy. Nested reverse transcriptase-polymerase chain reaction (RT-PCR), sequencing of the amplified alleles, and immunohistochemistry were performed to document the presence of enterovirus. The type of enterovirus was specified by indirect immunofluorescence assay. At the early phase of the disease, patients presented mild, non-specific viral symptoms, persistent unexplained fever, and pancytopenia. At the late phase, patients had more severe manifestations, such as persistent high fever, diarrhea, weight loss, hepatosplenomegaly, and hepatic dysfunction. The therapeutic approach consisted of supportive care, administration of immunoglobulin (400 mg/kg or 2 g/kg), and pleconaril. All patients had fatal outcome; two patients succumbed to multiorgan failure (MOF), while one patient succumbed to ventricular fibrillation. IAHS usually has fulminant course and leads to severe and life-threatening complications, such as liver failure and MOF. IAHS should always be included in the differential diagnosis of viral syndrome or unexplained fever. The therapeutic approach for IAHS should be administered as early as possible, before the progression to irreversible tissue damage. Early therapeutic intervention involving high doses of immunoglobulin might be beneficial for the patient's outcome.

Infectious Triggers of Hemophagocytic Syndrome in Children

Infectious Triggers of Hemophagocytic Syndrome in Children

Hemophagocytic syndrome in children: an important diagnostic consideration in fever of unknown origin.

To study the evolution of hemophagocytic syndrome (HPS) in children, we performed a retrospective review of 19 patients (median age, 17.4 months) in whom an infectious diseases consultation was requested at Texas Children's Hospital during the period of September 1991 through September 2001. Clinical findings consistent with HPS most frequently presented during days 6-14 of illness, concomitant with laboratory abnormalities. Fever was present for a median of 19 days before the diagnosis of HPS. Elevated serum lactate dehydrogenase and ferritin levels were noted in all patients. An infectious agent was identified in 42% of patients; 16% were found to have immunologic or vasculitic disease. HPS is a rare but often fatal disease that can initially present as fever of unknown origin with varying clinical findings, and it can be recognized by physicians who are familiar with the evolution of HPS. It is likely that many of these cases remain undiagnosed because of the HPS's rapidly fatal course.

Syndromes hémophagocytaires en phase d’aplasie post chimiothérapie

Material and methods. – This retrospective study reports 15 cases of hemophagocytic syndrome in children treated in our department during a eight-year period. Results. – Underlying diseases were acute lymphoblastic leukemia ( n=8) acute myeloblastic leukemia ( n=6) and Burkitt lymphoma ( n=1). Hemophagocytic syndrome was suspected after chemotherapy, in case of an unusual prolonged febrile neutropenia ( n=14) or isolated thrombocytopenia ( n=1). That fever was associated with cutaneous, pulmonary, hematologic, digestive and cardiac signs. Biological disorders included hypoprotidemia, hyponatremia, increased liver enzymes and fibrinopenia. Thrombocytopenia was observed in all patients and was associated with neutropenia for 14 of them. Diagnosis of hemophagocytic syndrome was always confirmed by bone marrow aspiration (infiltration with activated macrophages). Infection was documented in eight children. The treatment of hemophagocytic syndrome relied on steroids and resolution of symptoms occurred within three days of therapy. No recurrence of hemophagocytic syndrome was observed with a median follow up of two years and a half. Conclusion. – Such complication should be suspected in cases of prolonged febrile neutropenia and/or thrombocytopenia, and confirmed by bone marrow aspiration. Indeed, steroid therapy is effective and chemotherapy can be then pursued.

Hemophagocytic syndrome (HPS) in children and adults.

Hemophagocytic' syndrome (HPS) is a clinicopathologic entity characterized by systemic proliferation of benign hemophagocytic cells of the monocyte-macrophage-histiocyte lineage, associated with fever, cytopenias, hepatosplenomegaly, lymphadenopathy, and coagulopathy. Two forms of the syndrome have been well characterized; familial hemophagocytic lymphohistiocytosis (FHL) of infants and reactive hemophagocytic syndrome (RHS) encountered at any age. Although the clinical features and pathophysiology of HPS have been reported, the differences in the clinical characteristics of HPS in children and adults have not been studied extensively. In this article the latest concepts and clinical features of HPS are reviewed, focusing on the differences in features depending on the age of onset.