Anemia, Aplastic ; diagnosis ; pathology ; Humans ; Severity of Illness Index

OBJECTIVETo 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.

METHODSWe 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.

RESULTSThere 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).

CONCLUSIONRCC 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.

Adolescent ; Anemia, Aplastic ; diagnosis ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Myelodysplastic Syndromes ; diagnosis ; pathology ; Retrospective Studies

OBJECTIVETo explore the diagnosis and differential diagnosis of refractory cytopenia of children (RCC) according to WHO classification, and discuss the relationship between the cytology reviewed by hematologists and histology reviewed by pathologists.

METHODSWe selected 50 non-severe aplastic anemia cases from 2007 - 2010 in our hospital and collected clinical data. Experienced hematologists and pathologists evaluated bone marrow biopsy and smear respectively.

RESULTSOf 50 cases, 23 were male and 27 female (M:F = 1:1.17), the median age at diagnosis was 9 years (ranged from 3 to 14 years). 5 patients had disagreement of diagnosis between hematologists and pathologists. In 3 cases hematologists diagnosed as aplastic anemia (AA) and pathologists as RCC, 2 cases vice versa. The final diagnoses of 50 patients reached consensus between hematologists and pathologists were AA 16 cases, RCC 34 cases including 8 refractory cytopenias with multilineage dysplasia (RCMD) cases. All 16 cases AA showed severe hypocellularity. Only 4 cases (25.00%) RCC showed severe hypocellularity, 19 cases (73.08%) RCC showed mild hypocellularity and 3 cases (11.54%) RCC were normal hypocellularity.

CONCLUSIONOur results suggests that RCC was not rare in China. The main feature of RCC was dysplasia because of absence of increased blast. RCC was easily confused with AA. The main points of differential were present dysplastic changes of megakaryocyte best appreciated by the hematologists and morphologists and abnormal location of hematopoietic easily observed by pathologists. Overall, cytology and histology were complementary in the investigation of RCC and AA, because of sometimes one might give information that not be given from the other.

Adolescent ; Anemia, Aplastic ; diagnosis ; pathology ; Bone Marrow ; pathology ; Bone Marrow Examination ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Male ; Myelodysplastic Syndromes ; diagnosis ; pathology ; Pancytopenia ; diagnosis ; pathology ; Retrospective Studies

No abstract available.
Adolescent ; Anemia, Aplastic/*diagnosis/pathology ; Bone Marrow/pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Half-Life ; Humans ; Immunohistochemistry ; Male ; Mutation ; Myelodysplastic Syndromes/*diagnosis/pathology ; Retrospective Studies ; Tumor Suppressor Protein p53/genetics/*metabolism

We report two cases of adult acute lymphoblastic leukemia presenting with preleukemic phase of pancytopenia with a few abnormal lymphoid cells in bone marrow aspirates. The initial diagnosis of each case was suspicious aplastic anemia and hypoplastic anemia. Both cases progressed to overt acute lymphoblastic leukemia within 1 year. We suggest that initial pancytopenic phase (pre-ALL) may precede the diagnosis of acute lymphoblastic leukemia in adults and differential diagnosis from myelodysplastic syndrome and primary aplastic anemia will be needed. We also suggest that primary bone marrow lymphoma and "primary unknown metastatic lymphoma of bone marrow" may be possible as the pathogenesis in a case like ours.
Adult ; Anemia, Aplastic/diagnosis ; Bone Marrow/pathology ; Diagnosis, Differential ; Female ; Human ; Leukemia, Lymphocytic, Acute, L2/etiology* ; Leukemia, Lymphocytic, Acute, L2/diagnosis ; Male ; Myelodysplastic Syndromes/diagnosis ; Pancytopenia/etiology* ; Pancytopenia/diagnosis ; Preleukemia/etiology* ; Preleukemia/diagnosis

BACKGROUND: The presence of significant dysplasia in bone marrow (BM) aspirates helps to distinguish between hypocellular myelodysplastic syndrome (hMDS) and aplastic anemia (AA). Occasionally, diluted BM aspirates make it difficult to recognize dysplastic changes and can also negatively affect the detection of cytogenetic abnormalities in hMDS. We evaluated the usefulness of CD34 and p53 immunoreactivity for discriminating between hMDS and AA and for estimating survival outcomes in hMDS patients. METHODS: BM clot section (BMC) or BM biopsy (BMB) specimens were obtained from 64 hMDS/AA patients (33 with hMDS and 31 with AA) and seven controls. Immunohistochemical (IHC) staining for CD34 and p53 was performed by using the EnVision detection system (Dako, Denmark). We compared the results of IHC staining, BM findings, and chromosomal analyses, and determined overall survival outcomes. RESULTS: The number of CD34- and p53-positive BM cells was higher among the patients with hMDS than among the patients with AA (P<0.001 and P=0.001, respectively). hMDS patients with increased CD34-positive cells had significantly poorer survival outcomes compared with those with normal number of CD34-positive cells (P=0.013). CONCLUSIONS: CD34 and p53 IHC stains of BMC or BMB provide useful information for differentiating between hMDS and AA. CD34 IHC staining of BMC or BMB also provides useful information for estimating survival outcomes in hMDS patients.
Adolescent ; Adult ; Anemia, Aplastic/*diagnosis ; Antigens, CD34/*metabolism ; Bone Marrow/metabolism/*pathology ; Child ; Chromosome Aberrations ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Myelodysplastic Syndromes/*diagnosis/mortality ; ROC Curve ; Tumor Suppressor Protein p53/*metabolism

Behcet's disease is a multisystemic inflammatory disease characterized with recurrent oral ulcer, genital ulcer, and multiple organ involvement. Aplastic anemia is one of the rarest complications of Behcet's disease. There were only several reports about Behcet's disease associated myelodysplatic syndrome worldwide. Moreover, aplastic anemia in intestinal Behcet's disease was rarely reported. Here, we present a case of aplastic anemia with trisomy 8 and trisomy 9 in intestinal Behcet's disease and a review of the literatures. To the authors' knowledge, this is the first case ever reported in Korea.
Adult ; Anemia, Aplastic/complications/*diagnosis ; Behcet Syndrome/complications/*diagnosis/genetics ; Bone Marrow/pathology ; Chromosomes, Human, Pair 8 ; Chromosomes, Human, Pair 9 ; Female ; Humans ; Intestinal Diseases/complications/*diagnosis/genetics ; Karyotyping ; Tomography, X-Ray Computed ; *Trisomy

In order to investigate the significance of apoptosis and proliferation rates in differential diagnosis, evaluating curative effect and leukemia transformation in myelodysplastic syndromes, apoptosis index (AI) and proliferating index (PI) were assayed in marrow smears from 60 cases of MDS, 30 AML, 21 chronic aplastic anemia (CAA), 16 hemolytic anemia, 15 megaloblastic anemia and 30 normal controls. The apoptotic cells were assayed with TUNEL technique and proliferating cell nuclear antigen (PCNA) by immunohistochemical method in situ. The results showed that average AI in marrow smears from 39 cases with MDS prior therapy was (11.2 +/- 8.8)% and PI was (17.3 +/- 8.7)%, significant differences were observed in MDS group and normal control group, as well as in AML, CAA, megaloblastic anemia and hemolytic anemia groups. Hypoplastic MDS can be distinguished from CAA by AI and PI. Clinical therapy induced significant alteration of AI and PI in MDS, AML and CAA. After therapy of MDS, the AI dropped from (11.2 +/- 8.8)% to (6.6 +/- 0.7)%. It was concluded that examination of AI and PI of marrow cells in situ may provide valuable prognostic information, also can contribute to evaluate therapeutic effectiveness.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Anemia, Aplastic ; diagnosis ; Apoptosis ; Cell Division ; Child ; Child, Preschool ; Chronic Disease ; Female ; Humans ; In Situ Nick-End Labeling ; Male ; Middle Aged ; Myelodysplastic Syndromes ; diagnosis ; pathology

Abdominal Cavity ; Adult ; Anemia, Aplastic ; therapy ; Antigens, CD34 ; metabolism ; Carcinoma ; metabolism ; pathology ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Ki-67 Antigen ; metabolism ; Lymph Nodes ; pathology ; Male ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; etiology ; metabolism ; pathology ; Viral Proteins ; metabolism

Aplastic anemia is a rare complication of thymoma and is extremely infrequent after thymectomy. We present a case of a 60-year-old woman with very severe aplastic anemia appearing sixteen months after thymectomy for a thymoma. She underwent thymectomy for a thymoma in April 2000. Preoperative examination revealed no hematologic abnormality. About sixteen months after the operation, she was readmitted because of pancytopenia with cough and fever. Bone marrow aspiration revealed a very severe hypoplasia in all the three cell lines with over 80% fatty tissue, and chest CT revealed no recurrence of thymoma. Her aplastic anemia had responded to cyclosporine A and granulocyte-colony stimulating factor (G-CSF).
Anemia, Aplastic/drug therapy/*etiology/*pathology ; Biopsy, Needle ; Bone Marrow/pathology ; Cyclosporine/administration & dosage ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Granulocyte Colony-Stimulating Factor/administration & dosage ; Humans ; Middle Aged ; Rare Diseases ; Risk Assessment ; Severity of Illness Index ; Thymectomy/*adverse effects/methods ; Thymoma/diagnosis/*surgery ; Treatment Outcome