The study was aimed to investigate the clinical characteristics, diagnosis and therapy of patients with lymphoma associated hemophagocytic syndrome (LAHS) so as to provide the clinical basis for improving its recognition and giving effective therapy. The clinical data of 14 patients with LAHS in Beijing Friendship Hospital, Capital Medical University during the period from June 2005 to May 2008 were collected, the informations including primary diseases, clinical manifestations, laboratory findings, therapy and outcome were analyzed retrospectively, the coincidence of each diagnostic index was compared before and after diagnosis. All 14 patients were given therapeutic regimens containing fludarabine, methylprednisolone and gammaglobulin (FDIg) after final diagnosis. The results indicated that 100% patients had abnormal changes on NK cell activity and sCD25 level in serum, but hemophagocytosis in less than 40% patients at early stage was found in bone marrow. Even after confirmed diagnosis of the disease, the percentage of patients with hemophagocytosis was not up to 50%. 9 out of the 14 patients had a good prognosis after treatment, and the other 5 patients died. It is concluded that the detection of NK cell activity and sCD25 level in serum may be valuable for the early diagnosis of LAHS, the hemophagocytosis is not necessary for the diagnosis of LAHS. Fludarabine combined with methylprednisolone and gamma globulin may provide a new strategy for LAHS therapy.
Humans ; Lymphohistiocytosis, Hemophagocytic ; complications ; diagnosis ; drug therapy ; Lymphoma ; complications ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Vidarabine ; analogs & derivatives ; therapeutic use

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

We report a case of therapy-related acute myeloid leukemia after low-dosed topoisomerase II inhibitor (etoposide) treatment for hemophagocytic lymphohistiocytosis (HLH). A 62-yr-old female patient had previously been treated with a HLH-94 protocol containing a low-dose of etoposide (total dose of 300 mg/m2). Thirty-one months later, the patient was admitted to the hematology department with general weakness and upper respiratory infection symptoms. Peripheral blood smear and bone marrow study revealed acute monocytic leukemia. There was no evidence of myelodysplastic syndrome, and a cytogenetic study showed no chromosomal abnormalities.
Bone Marrow/pathology ; Etoposide/administration & dosage/*adverse effects ; Female ; Humans ; Leukemia, Monocytic, Acute/*chemically induced/*diagnosis/therapy ; Lymphohistiocytosis, Hemophagocytic/complications/*drug therapy ; Middle Aged

A case of hemophagocytic syndrome associated with ulcerative colitis is very rare. A 32-year-old man visited the hospital complaining of fever and severe abdominal pain for 7 days. He was diagnosed to have ulcerative colitis 2 years ago and had been treated with sulfasalazine. Three months ago, he had abdominal pain, weight loss, and hematochezia, so prednisolone and mercaptopurine were added to the treatment. On admission, the physical examination showed splenomegaly. Peripheral blood counts revealed pancytopenia, and bone marrow aspirate smears showed many histiocytes with active hemophagocytosis. There was no evidence of viral and bacterial infections and other neoplasms, which were commonly associated with hemophagocytic syndrome. He was successfully treated with high dose steroid. We report this case along with a review of the related literatures.
6-Mercaptopurine/therapeutic use ; Adult ; Anti-Inflammatory Agents/therapeutic use ; Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Bone Marrow Cells/pathology ; Colitis, Ulcerative/complications/*diagnosis/drug therapy ; Colonoscopy ; Dexamethasone/therapeutic use ; Humans ; Immunosuppressive Agents/therapeutic use ; Lymphohistiocytosis, Hemophagocytic/complications/*diagnosis/drug therapy ; Male ; Prednisolone/therapeutic use ; Sulfasalazine/therapeutic use ; Syndrome ; Tomography, X-Ray Computed

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