FEVER, PANCYTOPENIA, HEMOPHAGOCYTOSIS—CLINICAL ANALYSIS OF A CASE OF HEMATOLOGICAL SYSTEM DISEASE WITH BONE MARROW FAILURE AS THE PROMINENT MANIFESTATION
Keywords:
fever, pancytopenia, hemophagocytic syndromeAbstract
The object is to improve the recognition and differentiation of rare diseases of bone marrow failure. The method is to report a case with fever as the main symptom, pancytopenia, myelodysplasia and hemophagocytosis in the laboratory and to review literature. Results: The patient had fever for more than one month, no lymphadenopathy, but with hepatosplenomegaly, myelodysplasia with hemophagocytosis, EB virus infection. The hemogram recovered after 20 days of treatment. Conclusion: Hemophagocytic syndrome, acute aplastic crisis and severe aplastic anemia, the clinical manifestations can overlap. When the diagnosis is not very clear, the treatment should be based on strong supportive treatment, at the same time actively look for the causes, and remove the relevant factors.References
[1] . Henter JI, Horne AC, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis [J]. Pediatr Blood Cancer, 2007, 48(2):124-131. DOI:10.1002/pbc.21039.
[2] . Hemophagocytic syndrome Chinese expert alliance, hematology group of pediatric branch of Chinese Medical Association. Consensus of Chinese experts on diagnosis and treatment of hemophagocytic syndrome [J]. Chinese medical journal, 2018, 98(2):91-95.
[3] . Jordan MB, Hildeman D, Kappler J, et al. An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder [J]. Blood, 2004, 104(3):735-743.
[4] . Schram AM, Campigotto F, Mullally A, et al. Marked hyperferritinemia does not predict for HLH in the adult population [J]. Blood, 2015, 125(10):1548-1552.
[5] . Yin Xiaohua, Li Danhong, Wang Jiuwen, et al. Chronic aplastic anemia with hemophagocytic syndrome (a case report) [J]. Lymphoma, leukemia, 2002, 11(6):356-357.