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 investigate the clinical features of Pulmonary Langerhans' cells histiocytosis (PLCH). MethodsFour cases of PLCH diagnosed by histopathologic examination between August 2004 and September 2013 were retrospectively analyzed. ResultsFour male patients aged from 19 to 46 year old, including three smokers. The main symptoms were chest tightness, cough, and dyspnea. Pneumothorax was presented in two cases, and tuberculosis was in one. The chest high resolution CT (HRCT) revealed lung cysts, nodles, and reticular changes predominantly in the upper and middle lung fields. The pathological Langerhans' cells infiltration were found in the histological biopsy of lesions of the 4 cases. All of the patients were positive in the immuno-histological staining for the S-100 and CD1a antigens. Two cases were positive in Langrin staining (other two patients didn't underwent the staining). Two of the 4 patients were given oral steroid, and the symptoms were improved in one of them. The case with pulmonary tuberculosis improved in symptoms and CT results showed the absorption of the lesion after anti-tuberculosis therapy. Three cases were not followed up. ConclusionPLCH patients were mainly young adults, often presented with chest tightness, cough, and dyspnea. The clinical features of chest HRCT are bilateral cysts, nodules and reticular changes. The disease may be defined by the finding of pathologic Langerhans' cells or the positive staining for CD1a antigens or Langerin.
Objective To summarize the preliminary effectiveness of surgical treatment of Erdheim-Chester disease (ECD), so as to improve the understanding of the disease by orthopedic surgeons. Methods The clinical data of 9 patients with ECD between December 2012 and October 2017 were retrospectively analysed. There were 6 males and 3 females with an median age of 42 years (range, 8-61 years). The disease duration was 4-59 months (mean, 39 months). There were 2 cases of multiple lesions, including 1 case involving soft tissue of the buttocks and bilateral tibia, 1 case involving the sinus, skull base, and proximal right tibia; 7 cases with single lesion, including 3 cases of right femoral neck, 1 case of proximal right tibia, 1 case of right humerus, and 2 cases of ribs. Nine patients were diagnosed according to clinical manifestations, imaging examination, and pathological diagnosis. Four patients underwent needle biopsy before operation and 5 patients were diagnosed by postoperative pathology examination. Five cases underwent lesional scraping and internal fixation, 1 case underwent bone scraping and bone grafting, and 3 cases underwent lesion resection. One of the multiple lesions was treated with interferon and hormone. Results Nine patients underwent the surgery safely. There was no fever, wound exudation, infection, etc., and the incisions healed by first intention. All the patients were followed up 4-59 months with an average of 31.4 months. One patient with bilateral tibia and hip soft tissue involvement continued to receive medical treatment, and the tumor was controlled without significant increasing. The remaining 8 patients were examined for X-ray films at 3, 6, and 12 months after surgery, the bone has been fused and the steel plate and intramedullary nail were firmly fixed, and no tumor recurrence was observed. At 1 year after surgery, the pain symptoms of the patients improved and returned to normal life; 3 of them who involving the right femoral neck walked freely, and the quality of life improved significantly. Conclusion ECD patients can achieve the purpose of eliminating lesions and relieving pain after surgical treatment, and the surgical treatment has the advantages of quick relief of pain, improved quality of life, small side effects, and low economic cost when compared with medical treatment.