The acquired immunodeficiency syndrome patients with compromised immunity are prone to hemophagocytic syndrome secondary to opportunistic infections.This paper reports a rare case of hemophagocytic syndrome secondary to human parvovirus B19 infection in an acquired immunodeficiency syndrome patient,and analyzes the clinical characteristics,aiming to improve the diagnosis and treatment of the disease and prevent missed diagnosis and misdiagnosis.
Humans ; Lymphohistiocytosis, Hemophagocytic/drug therapy* ; Erythema Infectiosum/complications* ; Acquired Immunodeficiency Syndrome/complications* ; Parvoviridae Infections/diagnosis* ; Parvovirus B19, Human

OBJECTIVE: To investigate the clinical features of acute myeloid leukemia patients with hemophagocytic syndrome. METHODS: The clinical data of 2 patients with acute myeloid leukemia complicated with hemophagocytic syndrome were collected, and the clinical characteristics and treatment outcomes were analyzed. RESULTS: There were two patients with acute myeloid leukemia, including 1 male and 1 female，aged for 67 and 40 years old，respectively. Hemophagocytic syndrome occurred in one patient after induction therapy for acute myeloid leukemia and one patient after consolidation therapy. Both of the patients with hemophagocytic syndrome showed fever, hemocytopenia, high ferritin, high titer sCD25 levels and hemophagocytes in bone marrow. After achieved anti-infection, glucocorticoid, human immunoglobulin and etoposide regimens treatment, hemophagocytic syndrome was controlled in both of the two patients. One patient failed to induce acute myeloid leukemia and one patient achieved complete remission. CONCLUSION Acute myeloid leukemia complicated with hemophagocytic syndrome is rare. Early identification, early anti-infection combined with HLH94 regimen can control hemophagocytosis and improve prognosis.
Aged ; Antineoplastic Combined Chemotherapy Protocols ; Bone Marrow ; Female ; Humans ; Leukemia, Myeloid, Acute/drug therapy* ; Lymphohistiocytosis, Hemophagocytic/complications* ; Male ; Prognosis ; Treatment Outcome

Hemophagocytic syndrome (HPS) is a severe disease characterized by excessive release of inflammatory cytokines caused by abnormal activation of lymphocytes and macrophages, which can cause multiple organ damage and even death. Panniculitis is a disease characterized by inflammation of subcutaneous adipose tissue. We effectively treated 2 patients with panniculitis-associated HPS with ruxolitinib. Case 1: A 70-year-old male started with intermittent plantar swelling and pain, and then developed leukocytosis, mild anemia, multiple red maculopapules with painless subcutaneous nodules on the forehead, neck and bilateral lower legs. The patient was treated with prednisone and leflunomide for improvement. After that, repeated fever and rash occurred again. After admission to our hospital, we found his leukocyte and hemoglobin decreased, ferritin raised, fibrinogen and natural killer (NK) cell activity decreased, and hemophagocytic cells were found in bone marrow aspiration. The skin pathology was consistent with non-suppurative nodular panniculitis. He was diagnosed with nodular panniculitis associa-ted HPS. He was treated with glucocorticoid, cyclosporine, etoposide and gamma globule, but the disease was not completely controlled. After adjusting etoposide to ruxolitinib, his symptoms and abnormal laboratory findings returned to normal. After 2 months he stopped using ruxolitinib due to repeated infections. During the follow-up, though the prednisone dose was tapered, his condition was stable. Case 2: A 46-year-old female patient developed from intermittent fever, erythematous nodular rash with tenderness, leukopenia, and abnormal liver function. antibiotic therapy was ineffective. She improved after glucocorticoid treatment, and relapsed after glucocorticoid reduction. There were fever, limb nodules, erythema with ulcerative necrosis, intermittent abdominal pain when she came to our hospital. Blood examination showed that her white blood cells, red blood cells and platelets were decreased, fibrinogen was decreased, triglyceride was increased, ferritin and soluble interleukin-2 receptor(SIL-2R/sCD25) were significantly raised, and hemophagocytic cells were found in bone marrow aspiration. It was found that Epstein-Barr virus DNA was transiently positive, skin Staphylococcus aureus infection, and pulmonary Aspergillus flavus infection, but C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were normal, and no evidence of tumor and other infection was found. Skin pathology was considered panniculitis. The diagnosis was panniculitis, HPS and complicated infection. Antibiotic therapy and symptomatic blood transfusion were given first, but the disease was not controlled. Later, dexamethasone was given, and the condition improved, but the disease recurred after reducing the dose of dexamethasone. Due to the combination of multiple infections, the application of etoposide had a high risk of infection spread. Ruxolitinib, dexamethasone, and anti-infective therapy were given, and her condition remained stable after dexamethasone withdrawal. After 2 months of medication, she stopped using ruxolitinib. One week after stopping using ruxolitinib, she developed fever and died after 2 weeks of antibiotic therapy treatment in a local hospital. In conclusion, panniculitis and HPS are related in etiology, pathogenic mechanism and clinical manifestations. Abnormal activation of Janus-kinase and signal transduction activator of transcription pathway and abnormal release of inflammatory factors play an important role in the pathogenesis of the two diseases. The report suggests that ruxolitinib is effective and has broad prospects in the treatment of panniculitis associated HPS.
Humans ; Male ; Female ; Middle Aged ; Aged ; Lymphohistiocytosis, Hemophagocytic/drug therapy* ; Glucocorticoids/therapeutic use* ; Epstein-Barr Virus Infections/complications* ; Etoposide/therapeutic use* ; Prednisone/therapeutic use* ; Herpesvirus 4, Human ; Panniculitis/complications* ; Dexamethasone/therapeutic use* ; Exanthema/complications* ; Ferritins/therapeutic use* ; Anti-Bacterial Agents/therapeutic use* ; Fibrinogen/therapeutic use*

Child ; Epstein-Barr Virus Infections ; complications ; Female ; Giardia lamblia ; isolation & purification ; Giardiasis ; complications ; Herpesvirus 4, Human ; isolation & purification ; Humans ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; etiology

OBJECTIVETo analyze the clinical characteristics of Mycoplasma pneumoniae-associated hemophagocytic syndrome (MP-HLH).

METHODA retrospective investigation of the clinical manifestation, laboratory test, imagelogy, clinical course and outcome of 3 cases with MP-HLH seen between June 2013 and July 2013 in Shenzhen Children's Hospital, and review of relevant literature were conducted.

RESULTOf the 3 cases of MP-HLH, 2 were males, one was female, the ages were 1 year, 3 years and 6 years, respectively. They had no underlying disease previously. All the 3 cases had onset of fever, cough as main symptoms. Diagnosis of refractory Mycoplasma pneumoniae pneumonia was made, which was accompanied by decreased neutrophils [(0.08-0.68)×10(9)/L], hemoglobin [(79-103) g/L], platelet [(64-157)×10(9)/L], plasma fibrinogen [(1.3-1.5) g/L], lactate dehydrogenase [(1,170-1,285) U/L] and increased serum ferritin [(936.7-39 789.0) µg/L] in the third week of course. In two cases the T lymphocytes decreased, and the NK cell activity decreased significantly in one. Bone marrow cytology showed prompted bone marrow hyperplasia, and the phenomenon of phagocytosed blood cells. CT scan was performed for all the cases and consolidation with pleural effusion were shown. Two cases were admitted to PICU, and required endotracheal intubation and mechanical ventilation. Flexible bronchoscopy and bronchial lavage were performed and bronchial cast was found in two cases. All of them were treated with macrolide combined with other antibiotics, glucocorticoids and gamma globulin combination therapy, including one case given dexamethasone [10 mg/(m2·d)], cyclosporine[6 mg/(kg·d)], etoposide [150 mg/(m2·d)] chemotherapy. Two cases were cured, and 1 case died. The authors summarized the 18 cases reported in domestic and foreign literature. Foreign children were diagnosed and treated with steroids in 1-2 weeks, and 10 cases were cured, and 2 cases died. They died of massive hemorrhage and meningoencephalitis, and domestic children were diagnosed and treated within two to 4 weeks after onset, 5 cases were cured, one case died of severe pneumonia.

CONCLUSIONMP-HLH is a rare disease in children, and had acute onset, rapid progression and high mortality. Early treatment with steroids was associated with a good prognosis, the key to successful treatment is early diagnosis and treatment, avoiding the immune cascade. Too late a diagnosis or development of serious complications may lead to death.

Anti-Bacterial Agents ; therapeutic use ; Bronchoalveolar Lavage Fluid ; Bronchoscopy ; Child ; Child, Preschool ; Fatal Outcome ; Female ; Fever ; Glucocorticoids ; therapeutic use ; Humans ; Infant ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; drug therapy ; microbiology ; Macrolides ; therapeutic use ; Male ; Mycoplasma pneumoniae ; isolation & purification ; Pleural Effusion ; Pneumonia, Mycoplasma ; complications ; diagnosis ; drug therapy ; Respiration, Artificial ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo study the prognostic significance of coagulation disorders in children with hemophagocytic syndrome (HPS).

METHODSThirty-five children with HPS were retrospectively studied to analyze the etiology, clinical characteristics, laboratory results and treatment outcomes.

RESULTSAfter treatment, 27 of the 35 HPS patients survived, and the other 8 cases died. All cases were treated according to the HLH-2004 protocol, but etoposide (VP-16) was not used in 10 of them. The response rate in patients who received VP-16 (22/25, 88%) was significantly higher than that in those not receiving VP-16 (5/10, 50%) (P<0.05). Compared with the survival group, the dead group had significantly lower platelet count, fibrinogen level, and VP-16 utilization rate (P<0.05) but significantly longer activated partial thromboplastin time and prothrombin time (P<0.05).

CONCLUSIONSCoagulation function can be used as an indicator of disease outcome. It is essential for improving the clinical outcome of HPS to monitor the coagulation function during treatment, detect and correct abnormalities in time, and provide treatment strictly according to the HLH-2004 protocol.

Adolescent ; Child ; Child, Preschool ; Disseminated Intravascular Coagulation ; mortality ; Etoposide ; therapeutic use ; Female ; Humans ; Infant ; Lymphohistiocytosis, Hemophagocytic ; blood ; complications ; drug therapy ; mortality ; Male ; Prognosis ; Retrospective Studies

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and potentially fatal syndrome that results from inappropriate activation of lymphocytes and macrophages. It is characterized by fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, and pathologic findings of hemo- phagocytosis in the bone marrow or other tissues. We report an adult HLH case admitted to hepatology department.
Humans ; Liver Diseases ; complications ; physiopathology ; Liver Function Tests ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; etiology ; Male ; Middle Aged ; Prednisone ; therapeutic use

OBJECTIVETo explore the characteristics and risk of etoposide-related leukemia in the treatment of hemophagocytic lymphohistiocytosis (HLH).

METHODClinical characteristics of a case with secondary acute promyelocytic leukemia (APL) were summarized and 10 cases of secondary leukemia after treatment for HLH from literature were analyzed.

RESULTThe child was diagnosed with Epstein-Barr virus associated HLH and received HLH-2004 protocol. The cumulative dose of etoposide (VP16) was 3520 mg/m(2). The patient was diagnosed with APL after 28 months of HLH.He achieved complete remission after induction chemotherapy of all-trans-retinoic acid and darubicin. Consolidated chemotherapy was continued. There were 10 reports of etoposide-related leukemia after treatment for HLH in the literature.Review of 11 cases treated with VP16, of which cumulative doses were 900-20 500 mg/m(2). The interval period between HLH and secondary leukemia was 24 months. The types of secondary leukemia included 1 case with acute lymphoblastic leukemia, 1 case with myelodysplastic syndrome and 9 cases of acute myeloid leukemia. The abnormalities of chromosome included 3 patients with 11q23, 3 APL patients with t (15, 17).Seven patients survived and 4 died.

CONCLUSIONThe latency period of etoposide-related leukemia is short. Acute myeloid leukemia and balanced chromosomal abnormality are common in etoposide-related leukemia. The risk factors for development of secondary leukemia are related to cumulative drug doses of etoposide, treatment schedules and co-administration of other antineoplastic agents.It is appropriate to keep suitable range of the cumulative dose of etoposide in HLH therapy in order to reduce the risk of therapy related leukemia.

Acute Disease ; Antineoplastic Agents ; administration & dosage ; adverse effects ; Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Child, Preschool ; Daunorubicin ; administration & dosage ; Epstein-Barr Virus Infections ; complications ; drug therapy ; virology ; Etoposide ; administration & dosage ; adverse effects ; Humans ; Leukemia, Promyelocytic, Acute ; chemically induced ; drug therapy ; Lymphohistiocytosis, Hemophagocytic ; complications ; drug therapy ; virology ; Male ; Neoplasms, Second Primary ; chemically induced ; Risk Assessment ; Treatment Outcome ; Tretinoin ; administration & dosage

Hemophagocytic syndrome (HPS) is an uncommon hematological disorder that manifests as fever, splenomegaly, and jaundice, with hemophagocytosis in the bone marrow and other tissues pathologically. Secondary HPS is associated with malignancy and infection, especially viral infection. The prevalence of cytomegalovirus (CMV) infection in ulcerative colitis (UC) patients is approximately 16%. Nevertheless, HPS in UC superinfected by CMV is very rare. A 52-year-old female visited the hospital complaining of abdominal pain and hematochezia for 6 days. She was diagnosed with UC 3 years earlier and had been treated with sulfasalazine, but had stopped her medication 4 months earlier. On admission, her spleen was enlarged. The peripheral blood count revealed pancytopenia and bone marrow aspiration smears showed hemophagocytosis. Viral studies revealed CMV infection. She was treated successfully with ganciclovir. We report this case with a review of the related literature.
Antiviral Agents/therapeutic use ; Colitis, Ulcerative/*complications/drug therapy ; Cytomegalovirus Infections/*complications/drug therapy ; Female ; Ganciclovir/therapeutic use ; Gastrointestinal Agents/therapeutic use ; Gastrointestinal Hemorrhage/etiology ; Humans ; Lymphohistiocytosis, Hemophagocytic/drug therapy/*virology ; Middle Aged ; Sulfasalazine/therapeutic use ; Superinfection/*complications

Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening disorder due to hyperinflammation resulting in infiltration of different organs with extensive hemophagocytosis. Severe coagulopathy was one of the main reasons for death in HLH. Over secretion of plasminogen activator by activated macrophages leads to hyperfibrinolysis. We reported a 36-year-old woman who was diagnosed as HLH probably secondary to lymphoma. Massive bleeding from gut and retroperitoneal area were not able to be controlled by conventional hemostatic treatments. This patient received one dose recombinant activated factor VII (rFVIIa) 3.6 mg (70 μg/kg). Hemostatic effect was achieved in 0.5 hour and lasted 24 hours. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were quickly corrected to normal ranges. Fibrinogen level elevated from 0.5 g/L before using rFVIIa to 1.8 g/L 20 hours after. Although dexamethasone and etopside were administrated to treat HLH, this patient died from septic shock after persistent neutropenia. This suggests that rFVIIa may be effective in the management of intractable hemorrhage in patients with HLH.
Adult ; Disseminated Intravascular Coagulation ; complications ; Factor VIIa ; therapeutic use ; Female ; Humans ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; Recombinant Proteins ; therapeutic use