ObjectiveTo summarize the clinical manifestations,diagnosis and treatment of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis. MethodsOne proven diagnosed case of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis was analyzed. Related publications of case reports and articles from Wanfang Data and China Hospital Knowledge Database were also reviewed. ResultsA total of 15 cases were reported,in which 9 cases with complete clinical data. There were 7 males and 2 females,with a mean age of 31.2±15.83 years. The infected tissues were as follows:lung(6 cases),liver(2 cases),spleen(1 case),lymph node(2 cases),pleural(3 cases),peritoneum(2 cases),meninges(1 case),two or more sites infection(5 cases). The most clinical manifestations were fever,hepatosplenomegaly and enlarged lymph glands. The common laboratory abnormalities were liver dysfunction,pancytopaenia and haemophagocytosis in bone marrow. Treatment and outcomes:immunotherapy(5 cases,3 cases with intravenous immunoglobulin and 5 cases with steroid therapy) and anti-tuberculosis (14 cases). 3 cases were cured,6 cases improved and 6 died. ConclusionThere were no specific indexes of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis. Early proven diagnosis and administration of anti-tuberculosis and immunotherapy is essentials of effective therapy.
ObjectiveTo study the etiology, pathogenesis, clinical manifestations, diagnosis, treatment and prognosis of hemophagocytic syndrome (HPS). MethodsBetween February 2011 and October 2013, the causes, clinical manifestations, treatment and prognosis of 12 cases of HPS were retrospectively analyzed, and related literatures were reviewed. ResultsThere were 4 males and 8 females aged from 23 to 79 years old. Clinical features included fever (100.0%), lung infection (83.3%), enlarged lymph nodes (50.0%), bleeding (25.0%), splenomegaly (16.7%), and hepatomegaly (8.3%). Laboratory test results showed hemophagocytosis in bone marrow (100.0%), elevated ferritin (91.7%), thrombocytopenia (100.0%), decreased count in white blood cells (83.3%), decreased fibrinogen (66.7%), and hypertriglyceridemia (58.3%). Ten cases were related with infection, and 2 were related with diffused large B cell lymphoma. Nine patients died, 2 were cured, and one discharged himself from the hospital. ConclusionHPS is an uncommon disease, in which infection-associated HPS is the most common. The clinical presentation is complex, usually associated with multi-organ dysfunction, aggressive course, and high mortality rate.