scholarly journals Simultaneous Manifestation of Chronic Myelomonocytic Leukemia and Multiple Myeloma during Treatment by Prednisolone and Eltrombopag for Immune-Mediated Thrombocytopenic Purpura

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Masao Hagihara ◽  
Morihiro Inoue ◽  
Kenichiro Kodama ◽  
Tomoyuki Uchida ◽  
Jian Hua
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Chronic Myelomonocytic Leukemia ◽  
Severe Thrombocytopenia ◽  
Blood Monocytes ◽  
Thrombocytopenic Purpura ◽  
Peripheral Blood Monocytes ◽  
Immune Mediated ◽  
Dexamethasone Therapy ◽  
Myelomonocytic Leukemia

An 80-year-old man was admitted to our hospital because of severe thrombocytopenia. He was diagnosed with idiopathic thrombocytopenia, and prednisolone together with eltrombopag was started, leading to significant improvement of platelet counts. Four years later, there was a prominent increase of peripheral blood monocytes, which was accompanied by recurrence of thrombocytopenia. Bone marrow aspirates and serum electrophoresis revealed coexistence of chronic myelomonocytic leukemia (CMML) and multiple myeloma (MM). The patient received lenalidomide plus dexamethasone therapy but died due to exacerbation of the disorder. It was supposed that thrombocytopenia was secondarily caused by CMML and MM developed at a later period.

Chronic myelomonocytic leukemia developed 2 years after the onset of immune thrombocytopenic purpura like syndrome

Haematologia ◽  
2000 ◽  
Vol 30 (3) ◽  
pp. 221-224 ◽  
Author(s):  
Ali Uğur Ural ◽  
Cengiz Beyan ◽  
Kürşad Kaptan ◽  
Şefik Güran ◽  
Ferit Avcu ◽  
...  
Keyword(s):  
Immune Thrombocytopenic Purpura ◽  
Chronic Myelomonocytic Leukemia ◽  
Thrombocytopenic Purpura ◽  
Myelomonocytic Leukemia

Allogeneic bone marrow transplantation for chronic myelomonocytic leukemia in childhood: a report from the European Working Group on Myelodysplastic Syndrome in Childhood.

1997 ◽  
Vol 15 (2) ◽  
pp. 566-573 ◽  
Author(s):  
F Locatelli ◽  
C Niemeyer ◽  
E Angelucci ◽  
C Bender-Götze ◽  
S Burdach ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Myelodysplastic Syndrome ◽  
Marrow Transplantation ◽  
Working Group ◽  
Allogeneic Bone Marrow Transplantation ◽  
Chronic Myelomonocytic Leukemia ◽  
Allogeneic Bone ◽  
European Working ◽  
Myelomonocytic Leukemia

PURPOSE To evaluate the role of allogeneic bone marrow transplantation (BMT) in children with chronic myelomonocytic leukemia (CMML). PATIENTS AND METHODS Forty-three children with CMML given BMT and reported to the European Working Group on Myelodysplastic Syndrome in Childhood (EWOG-MDS) data base were evaluated. In 25 cases, the donor was a human leukocyte antigen (HLA)-identical or a one-antigen-disparate relative, in four cases a mismatched family donor, and in 14 a matched unrelated donor (MUD). Conditioning regimens consisted of total-body irradiation (TBI) and chemotherapy in 22 patients, whereas busulfan (Bu) with other cytotoxic drugs was used in the remaining patients. RESULTS Six of 43 patients (14%), five of whom received transplants from alternative donors, failed to engraft. There was a significant difference in the incidences of chronic graft-versus-host disease (GVHD) between children transplanted from compatible/one-antigen-mismatched relatives and from alternative donors (23% and 87%, respectively; P < .005). Probabilities of transplant-related mortality for children given BMT from HLA-identical/one-antigen-disparate relatives or from MUD/ mismatched relatives were 9% and 46%, respectively. The probability of relapse for the entire group was 58%, whereas the 5-year event-free survival (EFS) rate was 31%. The EFS rate for children given BMT from an HLA-identical sibling or one-antigen-disparate relative was 38%. In this latter group, patients who received Bu had a better EFS compared with those given TBI (62% v 11%, P < .01). CONCLUSION Children with CMML and an HLA-compatible relative should be transplanted as early as possible. Improvement of donor selection, GVHD prophylaxis, and supportive care are needed to ameliorate results of BMT from alternative donors.

scholarly journals Metastatic Breast Cancer with Extensive Osseous Metastasis Presenting with Symptomatic Immune Thrombocytopenic Purpura and Anemia: A Case Report and Review of the Literature

2015 ◽  
Vol 8 (2) ◽  
pp. 256-263 ◽  
Author(s):  
Jiaxin Niu ◽  
Teresa Goldin ◽  
Maurie Markman ◽  
Madappa N. Kundranda
Keyword(s):  
Breast Cancer ◽  
Platelet Count ◽  
Metastatic Breast Cancer ◽  
Immune Thrombocytopenic Purpura ◽  
English Literature ◽  
Metastatic Breast ◽  
Response To Therapy ◽  
Severe Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated

Background: Immune thrombocytopenic purpura (ITP) is a rare acquired bleeding disorder with an estimated incidence of 1 in 10,000 people in the general population. The association of ITP with breast cancer is an even rarer entity with very limited reports in the English literature. Case Presentation: We report a case of a 51-year-old female with no significant past medical history who presented with sudden onset of malaise, syncope, gingival bleed and epistaxis. She was found to have severe thrombocytopenia (platelet count 6,000/μl) and anemia (hemoglobin 7.2 g/dl). Her workup led to the diagnosis of metastatic ductal breast cancer with extensive bone metastasis. Bone marrow biopsy demonstrated myelophthisis which was initially thought to be consistent with her presentation of thrombocytopenia and anemia. Therefore, the patient was started on hormonal therapy for the treatment of her metastatic breast cancer. After 3 months of therapy, she did not improve and developed severe mucosal bleeding. Her clinical presentation was suspicious for ITP and immune-mediated anemia, and hence she was started on steroids and intravenous immunoglobulin. The patient had a dramatic response to therapy with normalization of her platelet count and hemoglobin within 2 weeks. Conclusion: To our knowledge, this is the first reported case of metastatic breast cancer presenting with symptomatic ITP and anemia, and both symptoms are postulated to be immune-mediated.

scholarly journals Bone marrow fibrosis in chronic myelomonocytic leukemia is associated with increased megakaryopoiesis, splenomegaly and with a shorter median time to disease progression

Oncotarget ◽  
2017 ◽  
Vol 8 (61) ◽  
pp. 103274-103282 ◽  
Author(s):  
Kseniya Petrova-Drus ◽  
April Chiu ◽  
Elizabeth Margolskee ◽  
Sharon Barouk-Fox ◽  
Julia Geyer ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Disease Progression ◽  
Median Time ◽  
Chronic Myelomonocytic Leukemia ◽  
Bone Marrow Fibrosis ◽  
Myelomonocytic Leukemia ◽  
Marrow Fibrosis

scholarly journals Clinical and Morphological Profile of Immune Thrombocytopenic Purpura in Children - A Five Year Study in a Paediatric Tertiary Health Care Centre of South India

2020 ◽  
Vol 7 (46) ◽  
pp. 2724-2729
Author(s):  
Ashida M. Krishnan ◽  
Deepthi Raj M.L ◽  
Priya V.S ◽  
Arya R.S
Keyword(s):  
Health Care ◽  
Bone Marrow ◽  
Immune Thrombocytopenic Purpura ◽  
Peripheral Blood ◽  
South India ◽  
P Value ◽  
Severe Thrombocytopenia ◽  
Care Centre ◽  
Health Care Centre ◽  
Thrombocytopenic Purpura

BACKGROUND Immune Thrombocytopenic Purpura (ITP) is one of the most commonly encountered disease in paediatric practice. Thorough clinical and morphological study of peripheral blood and bone marrow is required for confirming ITP. Clinicomorphological aspects of paediatric ITP is a less studied topic especially in developing countries like India. The objective was to study the clinical and morphological profile of paediatric cases of ITP. METHODS This is a 5-year record based retrospective study conducted in a paediatric tertiary health care centre in Kerala, South India. Data of all paediatric cases diagnosed as ITP including clinical presentation, clinical findings, blood counts, peripheral blood morphology, bone marrow morphology, and treatment response was collected and entered in SPSS software version 16.0 and analysed. For assessing correlation, chi-square test was used. RESULTS The age of children ranged from 3 months to 15 years. H/o viral fever was noted in 53 % cases. Cases which had moderate and severe thrombocytopenia were 74 % and 21 % respectively. Isolated thrombocytopenia was the most common peripheral blood picture observed with few cases showing coexisting eosinophilia and anaemia. All cases showed megakaryocyte proliferation in marrow with 9 % cases showing coexisting iron deficiency anaemia. Majority of cases showed rapid response to steroid / IVIG therapy and the response had no correlation with grade of thrombocytopenia (p value < 0.05). CONCLUSIONS Paediatric cases of ITP usually present following viral infections or vaccination, with worrisome bleeding episodes, petechiae, ecchymosis or purpura. KEYWORDS ITP, Paediatrics, Platelet Count, Thrombocytopenia, Vaccination

Chronic Myelomonocytic Leukemia With Abnormal Bone Marrow Eosinophils

2003 ◽  
Vol 127 (9) ◽  
pp. 1214-1216
Author(s):  
Jason Hyde ◽  
Tsieh Sun
Keyword(s):  
Bone Marrow ◽  
Eosinophil Count ◽  
Chronic Myelomonocytic Leukemia ◽  
Small Subset ◽  
Rare Entity ◽  
Abnormal Bone Marrow ◽  
Acute Myelomonocytic Leukemia ◽  
Peripheral Eosinophil ◽  
Myelomonocytic Leukemia

Abstract Chronic myelomonocytic leukemia with eosinophilia is a recently defined rare entity frequently associated with t(5;12)(q33;p13) translocation. It usually shows a peripheral eosinophil count greater than 1500/μL. However, the literature contains a small subset of cases in which the major manifestation is bone marrow eosinophilia. We report a case similar to that subset and discuss our finding that the immature eosinophils are identical to those seen in acute myelomonocytic leukemia with abnormal bone marrow eosinophils.

scholarly journals Cell-mediated suppression of megakaryocytopoiesis in acquired amegakaryocytic thrombocytopenic purpura

Blood ◽  
1986 ◽  
Vol 68 (3) ◽  
pp. 619-626 ◽  
Author(s):  
AM Gewirtz ◽  
MK Sacchetti ◽  
R Bien ◽  
WE Barry
Keyword(s):  
Bone Marrow ◽  
T Cells ◽  
T Lymphocytes ◽  
Colony Formation ◽  
Progenitor Cell ◽  
Mononuclear Cells ◽  
Platelet Glycoprotein ◽  
Severe Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Marrow Cells

Abstract Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a disorder of hematopoiesis characterized by severe thrombocytopenia due to a selective reduction or total absence of megakaryocytes in an otherwise normal-appearing bone marrow. Although the development of autoantibodies directed against cells in the megakaryocyte progenitor cell pool has been implicated in the pathogenesis of this disorder, cell-mediated suppression of megakaryocytopoiesis has not been described. Accordingly, we report two cases of AATP in which in vitro suppression of megakaryocyte colony formation by autologous ancillary marrow cells was demonstrable. Light-density bone marrow mononuclear cells (MNCs) obtained from both patients were either plated directly into plasma clot cultures, or after first being depleted by adherent monocytes (M phi) or T lymphocytes using standard methodologies. In some experiments, the depleted ancillary marrow cells were recovered for autologous co-culture studies with the MNCs from which they had been depleted. Megakaryocyte colony formation was detected in the cultures using an indirect immunofluorescence assay with a rabbit anti- human platelet glycoprotein antiserum. Removal of M phi (n = 6), or T lymphocytes (n = 4) from normal marrow MNCs had no apparent effect on colony formation. In contrast, depleting T lymphocytes from the MNCs of patient 1 significantly augmented megakaryocyte colony formation; a similar effect was observed after depleting M phi from the MNCs of patient 2. This observed augmentation in colony formation could be abrogated by autologous co-culture with the putative suppressor cell at effector cell/target cell ratios of 1:10 in the case of T lymphocytes or 1:5 in the case of M phi. Neither suppression nor stimulation of megakaryocyte colony formation was observed after culturing normal MNCs with autologous T cells (n = 4) or M phi (n = 3) at similar or greater ratios. We also observed inhibition of megakaryocyte colony formation after culturing normal MNCs in the presence of tissue culture medium conditioned by the M phi of patient 2. This effect was shown to be specific for megakaryocytes since this same conditioned medium had no significant effect on BFU-E and CFU-E-derived colony formation by autologous marrow mononuclear cells. These results suggest that: both T cells and M phi are capable of exerting a regulatory effect on the proliferation of human megakaryocyte progenitor cells (CFU-Meg); in the case of M phi, a soluble factor elaborated by these cells may be responsible for suppressing CFU-Meg growth; and aberrant ancillary cell- megakaryocyte progenitor cell interactions may lead to clinically significant disease.

Alpha-defensins secreted by dysplastic granulocytes inhibit the differentiation of monocytes in chronic myelomonocytic leukemia

Blood ◽  
2010 ◽  
Vol 115 (1) ◽  
pp. 78-88 ◽  
Author(s):  
Nathalie Droin ◽  
Arnaud Jacquel ◽  
Jean-Baptiste Hendra ◽  
Cindy Racoeur ◽  
Caroline Truntzer ◽  
...  
Keyword(s):  
Peripheral Blood ◽  
Human Peripheral Blood ◽  
Chronic Myelomonocytic Leukemia ◽  
Cellular Heterogeneity ◽  
Uridine Diphosphate ◽  
Blood Monocytes ◽  
Healthy Donors ◽  
Leukemic Clone ◽  
Macrophage Colony ◽  
Myelomonocytic Leukemia

Abstract Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic disorder that occurs in elderly patients. One of the main diagnostic criteria is the accumulation of heterogeneous monocytes in the peripheral blood. We further explored this cellular heterogeneity and observed that part of the leukemic clone in the peripheral blood was made of immature dysplastic granulocytes with a CD14−/CD24+ phenotype. The proteome profile of these cells is dramatically distinct from that of CD14+/CD24− monocytes from CMML patients or healthy donors. More specifically, CD14−/CD24+ CMML cells synthesize and secrete large amounts of alpha-defensin 1-3 (HNP1-3). Recombinant HNPs inhibit macrophage colony-stimulating factor (M-CSF)–driven differentiation of human peripheral blood monocytes into macrophages. Using transwell, antibody-mediated depletion, suramin inhibition of purinergic receptors, and competitive experiments with uridine diphosphate (UDP)/uridine triphosphate (UTP), we demonstrate that HNP1-3 secreted by CD14−/CD24+ cells inhibit M-CSF–induced differentiation of CD14+/CD24− cells at least in part through P2Y6, a receptor involved in macrophage differentiation. Altogether, these observations suggest that a population of immature dysplastic granulocytes contributes to the CMML phenotype through production of alpha-defensins HNP1-3 that suppress the differentiation capabilities of monocytes.

A Case of Acquired Amegakaryocytic Thrombocytopenia with Anti-c-mpl Autoantibody: Comparison with Idiopathic Thrombocytopenic Purpura

2019 ◽  
Vol 142 (4) ◽  
pp. 239-243
Author(s):  
Bora Son ◽  
Hee sue Park ◽  
Hye Sook Han ◽  
Hee Kyung Kim ◽  
Seung Woo Baek ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Rare Disease ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Immunosuppressive Treatment ◽  
Severe Bleeding ◽  
Severe Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Acquired Amegakaryocytic Thrombocytopenia

Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare disease that causes severe bleeding. The pathogenesis and treatment of AAMT have not yet been defined. We report the case of a 60-year-old woman diagnosed with AAMT, who presented with severe thrombocytopenia, gastroin­testinal bleeding, and significantly reduced bone marrow megakaryocytes. The patient was treated with methylprednisolone, cyclosporin, and intravenous immunoglobulin. After 2 weeks of treatment, her platelet count started to increase, and her bone marrow megakaryocyte count had normalized 3 months after diagnosis. At the time of diagnosis, the patient was seropositive for anti-c-mpl antibody but was seen to be seronegative once the platelet count recovered. In contrast, anti-c-mpl antibodies were not detected in the serum of 3 patients with idiopathic thrombocytopenic purpura. This case study suggests that anti-c-mpl antibody plays an important role in the development of AAMT, and that intensive immunosuppressive treatment is required for autoantibody clearance and recovery of megakaryocyte count.

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