- Email: [email protected]
P-084 Cytopenia with multilineage dysplasia
Poster Presentations – 12th International Symposium on Myelodysplastic Syndromes / Leukemia Research 37 S1 (2013) S1–S117
cytes 1900/mm3 , myelemia 3%, blasts 8%, hemoglobin 11,7g/dl, platelets: 251000/mm3 . Blast cells plus promonocytes accounted for 14% of the nucleated BM cells, leading to the diagnosis of CMML, subtype II according to the WHO classification. Materials and Methods: A treatment with 5-Azacytidine, 75 mg/m2 / day s/c 7 d/month was started. The patient received 6 cycles with good tolerance but without efficacy. BM aspiration showed persistence of major dysgranulopoiesis, the proportion of blasts was 22% suggestive of progression to acute myeloid leukemia. The BM karyotype was unchanged. The patient received supportive care and hydroxyurea only. Results: Further FISH analyse of BM cells confirmed that the t(9;12)(q22;p12) resulted in the chimeric fusion of TEL (ETV6) and SYK genes. In February 2011, the patient exhibited splenomegaly, night sweats,and at that time the blood count showed leucocytes 23400/mm3 with 34% of blasts. Compassionate Use of PKC412 (Midostaurin) at a dosage of 100mg per day and low doses of AraC s/c 10 d per month were delivered with the patient’s consent. The patient remained in stable disease but no hematological response was observed until August 2011. All therapies were stopped at the patient’s request and death occurred one month later. Conclusions: Poor efficacy of PKC412 in our case of CMML with t(9;12)(q22;p12) was probably due to a late initiation of treatment in a patient with blastic transformation. This very rare entity has been previously described but only one case was reported (Y. Kuno Blood 2001). Using a SYK inhibitor at the early stages of the disease could be helpful in this very rare condition, which seems closer to a myeloproliferative neoplasm than a typical CMML.
P-083 5q-syndrome or diamond blackfan anemia: The perplexing diagnostic puzzle of red cell aplasia A. Vlachos 1 , J.E. Farrar 2 , E. Atsidaftos 3, E. Muir 3 , A. Narla 4 , T.C. Markello 5 , S. Singh 1 , L. Blanc 1 , M. Landowski 6 , H.T. Gazda 7 , J.M. Liu 1 , S.R. Ellis 8 , R.J. Arceci 9 , B.L. Ebert 10 , D.M. Bodine 11 , J.M. Lipton 1 . 1 Hematology/Oncology and Stem Cell Transplantation, Feinstein Institute for Medical Research Cohen Children’s Medical Center, Manhasset NY, USA; 2 Pediatric Hematology/Oncology, Winthrop P. Rockefeller Cancer Institute, Little Rock AR, USA; 3 Pediatric Hematology/Oncology, Feinstein Institute for Medical Research, Manhasset NY, USA; 4 Hematology/Oncology, Boston Children’s Hospital, Boston MA, USA; 5 Medical Genetics Branch, National Human Genome Research Institute, Bethesda MD, USA; 6 Genetics and Program in Genomics, Boston Children’s Hospital, Boston MA, USA; 7 Genetics and Program in Genomics, Boston Children’s Hospital Harvard Medical School, Boston MA, USA; 8 Biochemistry & Molecular Biology, University of Louisville, Louisville KY, USA; 9 Pediatric Oncology, Sidney Kimmel CCC at Johns Hopkins University, Baltimore MD, USA; 10 Hematology, Brigham and Women’s Hospital Harvard Medical School, Boston MA, USA; 11 Genetics and Molecular Biology Branch, National Human Genome Research Institute, Bethesda MD, USA Background: 5q- syndrome is a rare, acquired macrocytic anemia with a female predominance and a mean age at diagnosis of 70 years. The erythroid deficiency results from RPS14 haploinsufficiency. Lenalidomide has been effective in ameliorating symptoms in ∼70% of patients. Diamond Blackfan Anemia (DBA), a rare, heritable red cell aplasia which usually presents in infancy, is characterized by mutations/deletions in ten ribosomal protein (RP) genes (not RPS14) and GATA1, results in haploinsufficiency and occurs in 60% of DBA patients. Introduction: Children with macrocytic anemia and red cell failure are often evaluated for DBA and other inherited bone marrow failure syndromes, and for somatic deletions in chromosomes 5 and 7.
S59
Purpose: To further screen children with red cell failure, who are not identified to have a genetic abnormality, for chromosomal deletions. Materials and Methods: Single Nucleotide Polymorphism (SNP) array genotyping was performed on whole blood and lineage-specific DNA. Regions of mosaic monosomy were identified by visual inspection of minor allele frequency plots. Results: An acquired internal deletion on chromosome 5q involving RPS14 was identified in two patients, ages 5 and 17 years, with red cell failure and macrocytic anemia. The bone marrow on both patients showed marked selective decrease in the erythroid series and they became red cell transfusion-dependent. Cytogenetic analysis at diagnosis was normal in both patients. In the first patient SNP array genotyping analysis identified mosaic monosomy in two regions on 5q, one that includes RPS14 (16.1Mb) and another centromeric (1.5Mb) region, with an estimated 64% monosomy in the larger region. This region includes the entire 5q- syndrome commonly deleted region (CDR) at 5q33. The deletion does not include the two regulatory phosphatases, CDC25c and PP2A, implicated in the lenalidomide response. This patient did however respond to lenalidomide and is now transfusion-independent. SNP array on the second patient revealed a smaller, mosaic deletion containing RPS14 (77% monosomy over 897Kb), which was undetectable by FISH and karyotype analysis, and is completely included within the 5q33 CDR. The patient underwent hematopoietic stem cell transplantation prior to the discovery of the 5q deletion. Conclusions: New paradigms such as SNP array genotyping for evaluating patients with potential germline or acquired genetic disorders can provide the ability to diagnose undiagnosed or misdiagnosed conditions. The use of this approach clarified the diagnosis of two patients with atypical presentations of red cell failure. The definitive diagnosis of 5q- syndrome in these patients demonstrates the overlapping characteristics of these two rare bone marrow failure syndromes.
P-084 Cytopenia with multilineage dysplasia X. Tong. Department of Hematology, Hematology Disease, Hangzhou, China Background: The revised classification of MDS issued by the WHO in 2008 is now widely accepted, and the Vienna MDS working conference of 2007 proposed a basic diagnostic standard. In practice, however, MDS is diagnosed on wider criteria than the absence of an elevated blast cell ratio (>0.05) in the bone marrow, and is classified as refractory cytopenia with unilineage dysplasia, refractory anemia with ringed sideroblasts, refractory cytopenia with multilineage dysplasia (MDS-RCMD), unclassified, or MDS with simple 5q syndrome; RCMD has the highest incidence Introduction: pathologic hematopoiesis can occur in many diseases, but there has been little research on whether there are differences between such diseases and MDS-RCMD, what relationships exist between pathologic phenomena seen on smears and bone marrow pathology and peripheral blood changes, and whether pathologic hematopoiesis affects the development of disease. We addressed these questions in this study. Purpose: We explored the correlation between cell morphology in refractory cytopenia with multilineage dysplasia (RCMD) and anemia. Cell morphology was recorded according to WHO criteria in RCMD patients and controls, and relevant laboratory data were collected. Materials and Methods: Patients: We retrospectively analyzed the cell morphology of MDS patients from 2007 to 2011, and collected relevant laboratory data. Cell morphologic analysis and histopathologic analysis. Results: See Table 1.
Poster Presentations – 12th International Symposium on Myelodysplastic Syndromes / Leukemia Research 37 S1 (2013) S1–S117
S60
Table 1. Types and mean numbers of dysplasia observed in RCMD patients and controls
Granulocyte series Erythrocyte series Megakaryocyte series
MA (mean, range)
HA (mean, range)
IDA (mean, range)
ITP (mean, range)
ICUS (mean, range)
RCMD (mean, range)
3 (1–6) 4 (2–7) 1 (0–2)
1 (0–4) 3 (2–6) 0 (0–2)
1 (0–4) 3 (1–5) 0 (0–1)
1 (0–2) 1 (0–4) 1 (0–3)
2 (0–3) 1 (0–3) 0 (0–1)
4 (0–7) 6 (1–11) 2 (0–4)
Table 2. Relationship between type of dysplasia in erythrocyte series and degree of anemia in RCMD
Table 2. Patient characteristics
Symptoms Sustained cytopenia Prior treatment exposures Hazardous exposures Peripheral blood: Flow cytometry: myeloblasts
A (mean, range) B (mean, range) C (mean, range) D (mean, range) RCMD patients Type of dysplasia
10 2 (0–5)
22 4 (1–8)
64 6 (3–11)
50 8 (4–11)
Conclusions: In RCMD patients, the number of types of dysplasia was greater than that of benign lesions in the controls and the severity of anemia was positively correlated with the number of types of erythroid-specific dysplasia. The incidence of abnormal localization of immature precursors (ALIP) was correlated with the number of types of granulocyte-specific but not with erythroid- or megakaryocyte-specific dysplasia. The incidence of ALIP was lower in patients with anemia than in those without, but its severity was not related to the incidence of anemia.
P-085 All that is not MDS is lost: Circulating peripheral blast of undetermined significance (CBUS) J. Sprandio 1 , A. Dulau Florea 2 , E.C. Besa 1 . 1 Medical Oncology, Thomas Jefferson University, Philadelphia, USA; 2 Hematopathology, Thomas Jefferson University, Philadelphia, USA Background: Myelodysplastic syndromes (MDS) are hematologic neoplasms thought to develop over many years and even decades without showing specific diagnostic abnormalities. Idiopathic Cytopenia of undetermined significance (ICUS) and idiopathic bone marrow dysplasia of uncertain significance (IDUS) have more recently been described and are felt to be a ’premalignant’ condition that may eventually progresses to hematologic neoplasms. ICUS describes cases in which cytopenias can be severe; however, only mild dysplasia may be seen on bone marrow biopsy. IDUS has been describe as having significant dysplasia in the bone marrow; however, only mild cytopenias. Neither fulfills the minimal criteria for a diagnosis of MDS. Introduction: We present a case of CBUS in a young patient without sustained cytopenia that does not meet the minimal criteria for a diagnosis of MDS. Purpose: Evaluate the prognostic implications of peripheral circulating blasts. Materials and Methods: Standard testing was performed for MDS including: peripheral blood, bone marrow, flow cytometry, cytogenetics, and FISH. Results: See Tables 1 and 2. Table 1. Minimal criteria for a diagnosis of MDS Criteria Absence of de novo acute myeloid leukemia cytogenetic abnormalities* And at least TWO of the following: Sustained unexplained cytopenia Dysplastic morphology in erythroid, granulocyte, or megakaryocytet Acquired, sustained clonal cytogenetic abnormality in hematopoietic cells Increased blasts in the marrow (>5%)
Patient
ICUS
IDUS
+
+
+
–
+
–
+
–
+
– –
– –
– –
Conclusions: At three years, he continues to have CBUS without progression or further diagnostic criteria of MDS. He will continue to
Patient
ICUS
IDUS
– – – – Circulating blasts (1–5%), basophilia (up to 17%) One population (2% of all cells): expressing CD13, CD22, CD33, CD38, and CD117 Second population (1% of all cells): expressed CD13, CD15, CD33, CD34, CD38, and HLA-DR
+/– + – – –
+/– – – – –
–
–
Bone marrow biopsy Dysplasia
+ – + (megakaryocyte, mild megaloblastic erythroid changes) Blast cell increase >5% – – – Cellularity 100% (increased myeloid to erythroid unknown unknown ratio) Ringed sideroblasts >15% – – +/– Reticulin fibrosis Mild – – Cytogenetics Normal 46, XY – – Manual differential 2% myeloblasts with normal unknown unknown phenotype Flow cytometry No abnormal populations unknown unknown Abnormal cytogenetics – – – MDS FISH panel* – – +/–
need close follow-up and evaluation. We believe there is an abnormality with the myeloblast’s adhesion proteins that makes them unable to anchor in the marrow which therefore allows them to be released into the periphery.
P-086 Refractory cytopenia with multilineage dysplasia: Cellular morphology, abnormal localization of immature precursors and anemia X.M. Tong, D.A.N. Shen, L.F. Zhu. Department of Hematology Disease, Hangzhou, China Background: Myelodysplastic syndrome (MDS) is a group of clonal disorders of hematopoietic stem cells with diverse cellular morphology, clinical and biological characteristics, and underlying genetic defects. Introduction: Pathologic hematopoiesis can occur in many diseases, but there has been little research on whether there are differences between such diseases and MDS-RCMD, what relationships exist between pathologic phenomena seen on smears and bone marrow pathology and peripheral blood changes, and whether pathologic hematopoiesis affects the development of disease. We addressed these questions in this study. Purpose: We explored the correlation between cell morphology in refractory cytopenia with multilineage dysplasia (RCMD) and anemia. Materials and Methods: Cell morphologic analysis:On bone marrow smears, 500 nucleated cells, 200 myeloid cells and 200 erythroid cells were observed and the proportions of cells with morphologic changes due to abnormal development were recorded. Two hundred white blood cells were observed on peripheral blood smears. Histopathologic analysis:Biopsy samples were taken from the posterior superior iliac spine. Ten percent formalin-fixed and paraffinembedded sections were examined with hematoxylin and eosin and Gomofi reticular fiber staining. Results: In RCMD patients, the number of types of dysplasia was greater than that of benign lesions in the controls and the severity of anemia was positively correlated with the number of types of erythroid-specific dysplasia. The incidence of abnormal localization of immature precursors (ALIP) was correlated with the num-
cytes 1900/mm3 , myelemia 3%, blasts 8%, hemoglobin 11,7g/dl, platelets: 251000/mm3 . Blast cells plus promonocytes accounted for 14% of the nucleated BM cells, leading to the diagnosis of CMML, subtype II according to the WHO classification. Materials and Methods: A treatment with 5-Azacytidine, 75 mg/m2 / day s/c 7 d/month was started. The patient received 6 cycles with good tolerance but without efficacy. BM aspiration showed persistence of major dysgranulopoiesis, the proportion of blasts was 22% suggestive of progression to acute myeloid leukemia. The BM karyotype was unchanged. The patient received supportive care and hydroxyurea only. Results: Further FISH analyse of BM cells confirmed that the t(9;12)(q22;p12) resulted in the chimeric fusion of TEL (ETV6) and SYK genes. In February 2011, the patient exhibited splenomegaly, night sweats,and at that time the blood count showed leucocytes 23400/mm3 with 34% of blasts. Compassionate Use of PKC412 (Midostaurin) at a dosage of 100mg per day and low doses of AraC s/c 10 d per month were delivered with the patient’s consent. The patient remained in stable disease but no hematological response was observed until August 2011. All therapies were stopped at the patient’s request and death occurred one month later. Conclusions: Poor efficacy of PKC412 in our case of CMML with t(9;12)(q22;p12) was probably due to a late initiation of treatment in a patient with blastic transformation. This very rare entity has been previously described but only one case was reported (Y. Kuno Blood 2001). Using a SYK inhibitor at the early stages of the disease could be helpful in this very rare condition, which seems closer to a myeloproliferative neoplasm than a typical CMML.
P-083 5q-syndrome or diamond blackfan anemia: The perplexing diagnostic puzzle of red cell aplasia A. Vlachos 1 , J.E. Farrar 2 , E. Atsidaftos 3, E. Muir 3 , A. Narla 4 , T.C. Markello 5 , S. Singh 1 , L. Blanc 1 , M. Landowski 6 , H.T. Gazda 7 , J.M. Liu 1 , S.R. Ellis 8 , R.J. Arceci 9 , B.L. Ebert 10 , D.M. Bodine 11 , J.M. Lipton 1 . 1 Hematology/Oncology and Stem Cell Transplantation, Feinstein Institute for Medical Research Cohen Children’s Medical Center, Manhasset NY, USA; 2 Pediatric Hematology/Oncology, Winthrop P. Rockefeller Cancer Institute, Little Rock AR, USA; 3 Pediatric Hematology/Oncology, Feinstein Institute for Medical Research, Manhasset NY, USA; 4 Hematology/Oncology, Boston Children’s Hospital, Boston MA, USA; 5 Medical Genetics Branch, National Human Genome Research Institute, Bethesda MD, USA; 6 Genetics and Program in Genomics, Boston Children’s Hospital, Boston MA, USA; 7 Genetics and Program in Genomics, Boston Children’s Hospital Harvard Medical School, Boston MA, USA; 8 Biochemistry & Molecular Biology, University of Louisville, Louisville KY, USA; 9 Pediatric Oncology, Sidney Kimmel CCC at Johns Hopkins University, Baltimore MD, USA; 10 Hematology, Brigham and Women’s Hospital Harvard Medical School, Boston MA, USA; 11 Genetics and Molecular Biology Branch, National Human Genome Research Institute, Bethesda MD, USA Background: 5q- syndrome is a rare, acquired macrocytic anemia with a female predominance and a mean age at diagnosis of 70 years. The erythroid deficiency results from RPS14 haploinsufficiency. Lenalidomide has been effective in ameliorating symptoms in ∼70% of patients. Diamond Blackfan Anemia (DBA), a rare, heritable red cell aplasia which usually presents in infancy, is characterized by mutations/deletions in ten ribosomal protein (RP) genes (not RPS14) and GATA1, results in haploinsufficiency and occurs in 60% of DBA patients. Introduction: Children with macrocytic anemia and red cell failure are often evaluated for DBA and other inherited bone marrow failure syndromes, and for somatic deletions in chromosomes 5 and 7.
S59
Purpose: To further screen children with red cell failure, who are not identified to have a genetic abnormality, for chromosomal deletions. Materials and Methods: Single Nucleotide Polymorphism (SNP) array genotyping was performed on whole blood and lineage-specific DNA. Regions of mosaic monosomy were identified by visual inspection of minor allele frequency plots. Results: An acquired internal deletion on chromosome 5q involving RPS14 was identified in two patients, ages 5 and 17 years, with red cell failure and macrocytic anemia. The bone marrow on both patients showed marked selective decrease in the erythroid series and they became red cell transfusion-dependent. Cytogenetic analysis at diagnosis was normal in both patients. In the first patient SNP array genotyping analysis identified mosaic monosomy in two regions on 5q, one that includes RPS14 (16.1Mb) and another centromeric (1.5Mb) region, with an estimated 64% monosomy in the larger region. This region includes the entire 5q- syndrome commonly deleted region (CDR) at 5q33. The deletion does not include the two regulatory phosphatases, CDC25c and PP2A, implicated in the lenalidomide response. This patient did however respond to lenalidomide and is now transfusion-independent. SNP array on the second patient revealed a smaller, mosaic deletion containing RPS14 (77% monosomy over 897Kb), which was undetectable by FISH and karyotype analysis, and is completely included within the 5q33 CDR. The patient underwent hematopoietic stem cell transplantation prior to the discovery of the 5q deletion. Conclusions: New paradigms such as SNP array genotyping for evaluating patients with potential germline or acquired genetic disorders can provide the ability to diagnose undiagnosed or misdiagnosed conditions. The use of this approach clarified the diagnosis of two patients with atypical presentations of red cell failure. The definitive diagnosis of 5q- syndrome in these patients demonstrates the overlapping characteristics of these two rare bone marrow failure syndromes.
P-084 Cytopenia with multilineage dysplasia X. Tong. Department of Hematology, Hematology Disease, Hangzhou, China Background: The revised classification of MDS issued by the WHO in 2008 is now widely accepted, and the Vienna MDS working conference of 2007 proposed a basic diagnostic standard. In practice, however, MDS is diagnosed on wider criteria than the absence of an elevated blast cell ratio (>0.05) in the bone marrow, and is classified as refractory cytopenia with unilineage dysplasia, refractory anemia with ringed sideroblasts, refractory cytopenia with multilineage dysplasia (MDS-RCMD), unclassified, or MDS with simple 5q syndrome; RCMD has the highest incidence Introduction: pathologic hematopoiesis can occur in many diseases, but there has been little research on whether there are differences between such diseases and MDS-RCMD, what relationships exist between pathologic phenomena seen on smears and bone marrow pathology and peripheral blood changes, and whether pathologic hematopoiesis affects the development of disease. We addressed these questions in this study. Purpose: We explored the correlation between cell morphology in refractory cytopenia with multilineage dysplasia (RCMD) and anemia. Cell morphology was recorded according to WHO criteria in RCMD patients and controls, and relevant laboratory data were collected. Materials and Methods: Patients: We retrospectively analyzed the cell morphology of MDS patients from 2007 to 2011, and collected relevant laboratory data. Cell morphologic analysis and histopathologic analysis. Results: See Table 1.
Poster Presentations – 12th International Symposium on Myelodysplastic Syndromes / Leukemia Research 37 S1 (2013) S1–S117
S60
Table 1. Types and mean numbers of dysplasia observed in RCMD patients and controls
Granulocyte series Erythrocyte series Megakaryocyte series
MA (mean, range)
HA (mean, range)
IDA (mean, range)
ITP (mean, range)
ICUS (mean, range)
RCMD (mean, range)
3 (1–6) 4 (2–7) 1 (0–2)
1 (0–4) 3 (2–6) 0 (0–2)
1 (0–4) 3 (1–5) 0 (0–1)
1 (0–2) 1 (0–4) 1 (0–3)
2 (0–3) 1 (0–3) 0 (0–1)
4 (0–7) 6 (1–11) 2 (0–4)
Table 2. Relationship between type of dysplasia in erythrocyte series and degree of anemia in RCMD
Table 2. Patient characteristics
Symptoms Sustained cytopenia Prior treatment exposures Hazardous exposures Peripheral blood: Flow cytometry: myeloblasts
A (mean, range) B (mean, range) C (mean, range) D (mean, range) RCMD patients Type of dysplasia
10 2 (0–5)
22 4 (1–8)
64 6 (3–11)
50 8 (4–11)
Conclusions: In RCMD patients, the number of types of dysplasia was greater than that of benign lesions in the controls and the severity of anemia was positively correlated with the number of types of erythroid-specific dysplasia. The incidence of abnormal localization of immature precursors (ALIP) was correlated with the number of types of granulocyte-specific but not with erythroid- or megakaryocyte-specific dysplasia. The incidence of ALIP was lower in patients with anemia than in those without, but its severity was not related to the incidence of anemia.
P-085 All that is not MDS is lost: Circulating peripheral blast of undetermined significance (CBUS) J. Sprandio 1 , A. Dulau Florea 2 , E.C. Besa 1 . 1 Medical Oncology, Thomas Jefferson University, Philadelphia, USA; 2 Hematopathology, Thomas Jefferson University, Philadelphia, USA Background: Myelodysplastic syndromes (MDS) are hematologic neoplasms thought to develop over many years and even decades without showing specific diagnostic abnormalities. Idiopathic Cytopenia of undetermined significance (ICUS) and idiopathic bone marrow dysplasia of uncertain significance (IDUS) have more recently been described and are felt to be a ’premalignant’ condition that may eventually progresses to hematologic neoplasms. ICUS describes cases in which cytopenias can be severe; however, only mild dysplasia may be seen on bone marrow biopsy. IDUS has been describe as having significant dysplasia in the bone marrow; however, only mild cytopenias. Neither fulfills the minimal criteria for a diagnosis of MDS. Introduction: We present a case of CBUS in a young patient without sustained cytopenia that does not meet the minimal criteria for a diagnosis of MDS. Purpose: Evaluate the prognostic implications of peripheral circulating blasts. Materials and Methods: Standard testing was performed for MDS including: peripheral blood, bone marrow, flow cytometry, cytogenetics, and FISH. Results: See Tables 1 and 2. Table 1. Minimal criteria for a diagnosis of MDS Criteria Absence of de novo acute myeloid leukemia cytogenetic abnormalities* And at least TWO of the following: Sustained unexplained cytopenia Dysplastic morphology in erythroid, granulocyte, or megakaryocytet Acquired, sustained clonal cytogenetic abnormality in hematopoietic cells Increased blasts in the marrow (>5%)
Patient
ICUS
IDUS
+
+
+
–
+
–
+
–
+
– –
– –
– –
Conclusions: At three years, he continues to have CBUS without progression or further diagnostic criteria of MDS. He will continue to
Patient
ICUS
IDUS
– – – – Circulating blasts (1–5%), basophilia (up to 17%) One population (2% of all cells): expressing CD13, CD22, CD33, CD38, and CD117 Second population (1% of all cells): expressed CD13, CD15, CD33, CD34, CD38, and HLA-DR
+/– + – – –
+/– – – – –
–
–
Bone marrow biopsy Dysplasia
+ – + (megakaryocyte, mild megaloblastic erythroid changes) Blast cell increase >5% – – – Cellularity 100% (increased myeloid to erythroid unknown unknown ratio) Ringed sideroblasts >15% – – +/– Reticulin fibrosis Mild – – Cytogenetics Normal 46, XY – – Manual differential 2% myeloblasts with normal unknown unknown phenotype Flow cytometry No abnormal populations unknown unknown Abnormal cytogenetics – – – MDS FISH panel* – – +/–
need close follow-up and evaluation. We believe there is an abnormality with the myeloblast’s adhesion proteins that makes them unable to anchor in the marrow which therefore allows them to be released into the periphery.
P-086 Refractory cytopenia with multilineage dysplasia: Cellular morphology, abnormal localization of immature precursors and anemia X.M. Tong, D.A.N. Shen, L.F. Zhu. Department of Hematology Disease, Hangzhou, China Background: Myelodysplastic syndrome (MDS) is a group of clonal disorders of hematopoietic stem cells with diverse cellular morphology, clinical and biological characteristics, and underlying genetic defects. Introduction: Pathologic hematopoiesis can occur in many diseases, but there has been little research on whether there are differences between such diseases and MDS-RCMD, what relationships exist between pathologic phenomena seen on smears and bone marrow pathology and peripheral blood changes, and whether pathologic hematopoiesis affects the development of disease. We addressed these questions in this study. Purpose: We explored the correlation between cell morphology in refractory cytopenia with multilineage dysplasia (RCMD) and anemia. Materials and Methods: Cell morphologic analysis:On bone marrow smears, 500 nucleated cells, 200 myeloid cells and 200 erythroid cells were observed and the proportions of cells with morphologic changes due to abnormal development were recorded. Two hundred white blood cells were observed on peripheral blood smears. Histopathologic analysis:Biopsy samples were taken from the posterior superior iliac spine. Ten percent formalin-fixed and paraffinembedded sections were examined with hematoxylin and eosin and Gomofi reticular fiber staining. Results: In RCMD patients, the number of types of dysplasia was greater than that of benign lesions in the controls and the severity of anemia was positively correlated with the number of types of erythroid-specific dysplasia. The incidence of abnormal localization of immature precursors (ALIP) was correlated with the num-