title: [Refractory cytopenia of children and acquired aplastic anemia: a clinical and pathological study of 130 cases].
abstract: OBJECTIVE
  To explore the clinical characteristics and histopathological morphology features of bone marrow biopsies between refractory cytopenia of children (RCC) and acquired aplastic anemia (AAA) to facilitate the diagnosis, differential diagnosis and treatment of RCC and AAA.
  
  
  METHODS
  We retrospectively analyzed clinical data and histopathological morphology of bone marrow biopsies in RCC or AAA patients referred to our hospital from January 2011 to December 2012.
  
  
  RESULTS
  There were totally 130 patients studied. The final diagnoses of them were RCC in 78 cases (60.0%) and AAA in 52 cases (40.0%). The median WBC count, absolute neutrophil count, blood platelet count, hemoglobin level, and reticulocyte count were all higher in RCC children than AAA (P<0.01). All of RCC patients showed hypocellular biopsy specimens, and 84.6% (66/78) of them had cellularity of bone marrow biopsy specimens ranging from 20% to 60%. Patchy pattern distribution was seen in 98.7% (77/78) of RCC cases, and micromegakaryocyte was found in 61.5% (48/78) of RCC cases. All of AAA patients showed severe hypocellular biopsy specimens, and 88.5% (46/52) of them had cellularity of bone marrow biopsy specimens under 5%. Megakaryocyte was not found in 98.1% (51/52) of AAA cases. The response rates of immunosuppressive therapy using CsA ± rabbit anti- thymocyte globulin ± androgen ± traditional Chinese medicine for patients with RCC and AAA were 59.5% and 26.9% at 3 months (P=0.011), and 75.0% and 38.1% at 6 months, respectively (P=0.007).
  
  
  CONCLUSIONS
  RCC patients showed milder cytopenia and bone marrow hyperplasia than AAA. Patchy distribution of hematopoietic cells, erythroid islands with a marked left shift and micromegakaryocytes were decisive histomorphological patterns used to separate RCC from SAA. Immunosuppressive therapy using CsA ± rabbit anti- thymocyte globulin ± androgen ± traditional chinese medicine was an effective therapy in patients with RCC and AAA, and the outcome of immunosuppressive therapy for RCC patients was superior to that of AAA patients.
authors.affiliation: Department of Pathology, Shanghai Children's Medical Center affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.
authors.forename: Xia
authors.initials: X
authors.lastname: Qin
authors.affiliation: Department of Pathology, Boeblingen Hospital, Clinical Centre South West.
authors.forename: Irith
authors.initials: I
authors.lastname: Baumann
authors.affiliation: Department of Pathology, Shanghai Children's Medical Center affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Chen
authors.affiliation: Department of Pathology, Shanghai Children's Medical Center affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.
authors.forename: Ping
authors.initials: P
authors.lastname: Shen
authors.affiliation: Department of Pathology, Shanghai Children's Medical Center affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.
authors.forename: Jiefeng
authors.initials: J
authors.lastname: Chen
authors.affiliation: Department of Pathology, Shanghai Children's Medical Center affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.
authors.forename: Minzhi
authors.initials: M
authors.lastname: Yin
mesh_terms: D000293:Adolescent; D000741:Anemia, Aplastic / Q000175:diagnosis* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000473:pathology; D012189:Retrospective Studies