scholarly journals Prognostic impact of bone marrow fibrosis and dyserythropoiesis in autoimmune hemolytic anemia

2018 ◽  
Vol 93 (4) ◽  
pp. E88-E91 ◽  
Author(s):  
Bruno Fattizzo ◽  
Anna Zaninoni ◽  
Umberto Gianelli ◽  
Alberto Zanella ◽  
Agostino Cortelezzi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Marrow Fibrosis

Imatinib reduces bone marrow fibrosis and overwhelms the adverse prognostic impact of reticulin formation in patients with chronic myeloid leukaemia

2016 ◽  
Vol 69 (9) ◽  
pp. 810-816 ◽  
Author(s):  
Eda Tanrikulu Simsek ◽  
Ahmet Emre Eskazan ◽  
Mahir Cengiz ◽  
Muhlis Cem Ar ◽  
Seda Ekizoglu ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Chronic Myeloid Leukaemia ◽  
Myeloid Leukaemia ◽  
Imatinib Therapy ◽  
Imatinib Treatment ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Significant Difference ◽  
Grade Ii ◽  
Marrow Fibrosis

AimsBefore the era of tyrosine kinase inhibitors (TKIs), the presence of bone marrow fibrosis (MF) in patients with chronic myeloid leukaemia (CML) has been established as a poor prognostic factor. The aim of the present study was to evaluate the effects of imatinib treatment on MF and the prognostic significance of MF at this new era of CML therapy.MethodsThe study cohort consisted of 135 patients with CML who were exposed to imatinib. The grades of MF pre and post imatinib together with cytogenetic and molecular responses were evaluated.ResultsSevere MF (grade II–III) was observed in 44 (33%) patients prior to imatinib therapy, and in 8 (8%) after 12 months of imatinib treatment (p=0.001). The complete cytogenetic response (CCyR) rates at 12 months did not differ according to the pre-imatinib MF grades, and CCyR rates in patients with grades 0, I, II and III MF were 36/47 (76.5%), 26/33 (78.7%), 12/23 (52.1%) and 7/10 (70%), respectively (p=0.127). There was no significant difference between patients with or without CCyR at 12 months of imatinib regarding grades of MF (p=0.785). The distribution of the major molecular response rates at 18 months according to pre-treatment grades of MF were determined as grade 0 in 38/45 (84.4%), grade I in 21/28 (75%), grade II in 14/21 (66.6%) and grade III in 7/10 (70%) (p=0.112). There was no significant difference in overall survival rates between initial MF mild (grade 0–I) and severe (grade II–III) groups (p=0.278).ConclusionsAccording to our findings, MF regresses with imatinib therapy over time, and the MF grades at diagnosis do not have a negative impact on the responses to imatinib treatment. Therefore, the adverse prognostic impact of the MF among patients with CML seems to disappear in the era of the TKIs.

scholarly journals Autoimmune myelofibrosis associated with autoimmune hemolytic anemia successfully treated with ruxolitinib

2021 ◽  
Author(s):  
Shuku Sato ◽  
Wataru Kamata ◽  
Yotaro Tamai
Keyword(s):  
Bone Marrow ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Transforming Growth Factor ◽  
Myeloproliferative Neoplasms ◽  
Bone Marrow Examination ◽  
Coombs Test ◽  
Bone Marrow Fibrosis ◽  
Cytokine Levels ◽  
Antiglobulin Test

Abstract A 55-year-old man suffered from dyspnea, general malaise, and jaundice. His laboratory date showed pancytopenia and hemolytic anemia, and computed tomography showed splenomegaly. Bone marrow examination revealed myelofibrosis (MF)-1. The hemolytic anemia was diagnosed as IgM autoimmune hemolytic anemia (AIHA) with negative direct and indirect Coombs test but positive IgM-direct antiglobulin test. We started ruxolitinib 20 mg, which improved not only bone marrow fibrosis, symptoms related to myeloproliferative neoplasms and splenomegaly, but also AIHA. AIHA may be associated with Autoimmune MF (AIMF), and cytokines such as transforming growth factor (TGF)-β are thought to be involved in such cases. This case suggests that ruxolitinib may improve the cytokine levels and may lead to the treatment of AIHA as well as AIMF.

P112 Prognostic impact of bone marrow fibrosis in myelodysplastic syndromes (MDS)

2007 ◽  
Vol 31 ◽  
pp. S100
Author(s):  
S. Baghikar ◽  
S. Braunstein ◽  
P. Reinecke ◽  
S. Knipp ◽  
R. Haas ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Myelodysplastic Syndromes ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Marrow Fibrosis

The prognostic impact of bone marrow fibrosis in primary myelofibrosis

2016 ◽  
Vol 91 (10) ◽  
pp. E454-E455 ◽  
Author(s):  
Paola Guglielmelli ◽  
Alessandro M. Vannucchi ◽  
Keyword(s):  
Bone Marrow ◽  
Primary Myelofibrosis ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Marrow Fibrosis

Prognostic Impact of Bone Marrow Fibrosis in Polycythemia Vera: Validation of the IWG-MRT Study and Additional Observations

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3129-3129
Author(s):  
Daniela Barraco ◽  
Sonia Cerquozzi ◽  
Curtis A. Hanson ◽  
Rhett P. Ketterling ◽  
Animesh Pardanani ◽  
...  
Keyword(s):  
Risk Factors ◽  
Bone Marrow ◽  
Polycythemia Vera ◽  
Univariate Analysis ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Abnormal Karyotype ◽  
Free Survival ◽  
Who Criteria ◽  
Marrow Fibrosis

Abstract Background Increased bone marrow (BM) reticulin fibrosis in polycythemia vera (PV) has been reported in 20% (Ann Hematol. 1999;78:495) to 51% (Eur J Haematol. 2011;86:148) of patients at diagnosis. In a previous report by the international working group for myeloproliferative neoplasms (MPN) research and treatment (IWG-MRT), the presence of BM fibrosis (≥ grade 1) at diagnosis was associated with a lower incidence of thrombosis during the clinical course and a higher risk of fibrotic progression while it did not affect overall (OS) or leukemia-free (LFS) survival (Blood. 2012;119:2239). The objectives for the current single center study of 262 PV patients were to validate the observations from the IWG-MRT and also identify other risk factors for myelofibrosis-free survival (MFFS) in PV. Methods Study patients were selected from our institutional database of MPN and fulfilled the 2016 World Health Organization (WHO) criteria for the diagnosis of PV (Blood. 2016;127:2391). Cytogenetic analysis and reporting was done according to the International System for Human Cytogenetic Nomenclature (Cytogenetic and Genome Research 2013;141:1-6). The degree of BM reticulin fibrosis was based on "real life" BM reports from Mayo Clinic hematopathologists and often in accordance with the European consensus scoring system (Haematologica 2005;90:1128). Statistical analyses considered clinical and laboratory parameters obtained at time of diagnosis. Results Patient characteristics: Analysis was conducted on 262 patients (median age 62 years; 50% males) who met 2016 WHO criteria for diagnosis of PV. Median (range) values were: hemoglobin 18 g/dl (14.8-24), leukocyte count 11.7 x109/L (4.3-59.3) and platelet count 454 x109/L (44-2747). Among informative cases, palpable splenomegaly was present in 27%, pruritus in 33% and erythromelalgia in 6%. Thrombosis history at diagnosis was documented in 28% of the patients and 23% experienced thrombotic events after diagnosis. Information on cytogenetics was available in 142 patients and karyotype was abnormal in 19%. BM reticulin fibrosis was reported to be absent in 135 patients (MF-0, 52%), grade 1 (MF-1) in 101 (39%), grade 2 (MF-2) in 22 (8%) and grade 3 (MF-3) in 4 (2%) patients. After a median follow up of 85 months, 107(41%) deaths, 30 (11%) fibrotic progression and 5 (2%) leukemic transformations were documented. Comparison of patients with and without bone marrow fibrosis A number of clinical and laboratory parameters were evaluated for possible association with the presence of ≥ grade 1 BM reticulin fibrosis and none, including age, sex, complete blood count, palpable splenomegaly, pruritus or erythromelalgia displayed a significant association. In univariate analysis, the presence of BM fibrosis (MF-0 versus MF-1 or greater) did not affect OS (p=0.5), LFS (p=0.2) or thrombosis-free survival (p=0.97) whereas a significant association was noted for MFFS (p=0.009; HR 2.9, 95% CI 1.3-6.7). Others risk factors for MFFS, in a univariate analysis, were leukocytosis ≥15 x 109/L (p=0.02; HR 2.7, 95% CI 1.17-6.48), presence of splenomegaly (p=0.02; HR 2.6, 95% CI 1.16-6) and abnormal karyotype (p=0.0005; HR 4.6, 95% CI 1.9-11). During multivariable analysis, not including karyotype, leukocytosis ≥15 x 109/L (p=0.04), presence of splenomegaly (p=0.04) and presence of BM reticulin fibrosis (p=0.01) remained significant; however, this significance for leukocytosis ≥15 x 109/L, presence of splenomegaly and BM reticulin fibrosis was lost when abnormal karyotype was added as covariate to each risk factor individually (p=0.4, p=0.1 and p=0.9, respectively). Conclusion We report a not infrequent (48% incidence) occurrence of ≥ grade 1 BM reticulin fibrosis at time of initial diagnosis of PV. In the current study, we did not find a prognostic impact for the presence of BM reticulin fibrosis, in terms of OS, LFS or thrombosis-free survival; however, a significant association was confirmed for MFFS that was independent of other non-genetic risk factors. The preliminary observation on the adverse prognostic impact of abnormal karyotype on MFFS requires additional studies. Disclosures No relevant conflicts of interest to declare.

scholarly journals Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations

2017 ◽  
Vol 7 (3) ◽  
pp. e538-e538 ◽  
Author(s):  
D Barraco ◽  
S Cerquozzi ◽  
C A Hanson ◽  
R P Ketterling ◽  
A Pardanani ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Polycythemia Vera ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Marrow Fibrosis

Clinical Significance of Bone Marrow Fibrosis in Patients with Polycythemia Vera

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5149-5149
Author(s):  
Aziz Nazha ◽  
Jorge E. Cortes ◽  
Zeev Estrov ◽  
Sherry Pierce ◽  
Hagop M. Kantarjian ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Leukemia ◽  
Polycythemia Vera ◽  
Clinical Manifestation ◽  
World Health ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Md Anderson ◽  
The Impact ◽  
Marrow Fibrosis

Abstract Abstract 5149 Background: Polycythemia vera (PV) is a heterogeneous myeloproliferative disease characterized by expansion of morphologically normal red blood cells, granulocytes, and platelets with varying degrees of bone marrow fibrosis (BMF) during the disease course. BMF as a result of abnormal deposition of reticulin and collagen fibers in bone marrow stroma plays a role in the pathophysiology and clinical manifestation of myeloproliferative disorders in general. However, in PV, older age and previous history of thrombosis remain the only two major risk factors for consideration in decisions regarding therapy. Objectives: To investigate the characteristics associated with BMF and its prognostic impact on clinical manifestation, overall survival (OS), and transformation to primary myelofibrosis and acute leukemia in patients with PV. Methods: We conducted a retrospective chart review analysis of 115 patients who were diagnosed with PV according to World Health Organization criteria and were referred to MD Anderson Cancer Center between May 2000 and December 2009. Results of the first bone marrow biopsy done at MD Anderson were reviewed. BMF was documented according to the European consensus grading system (MF0-3), in which MF-3 is the most severe grade of fibrosis. Results: Of the 115 patients, 23 (20%) had MF-0, 46 (40%) MF-1, 36 (31%) MF-2, and 10 (9%) MF-3. Table 1 summarizes patient characteristics and outcomes by grade. Conclusion: Severe BMF was associated with higher risk of bleeding and thrombosis and larger spleen in patients with PV. There was no association between BMF severity and the demographic or symptoms of the disease. There was no association between BMF severity and the presence of JAK2 mutation or cytogenetic abnormalities. There was no impact of BMF on OS, EFS, or transformation to myelofibrosis or acute leukemia. However, longer follow-up is needed to investigate further the impact of BMF on OS and transformation-free survival. Disclosures: No relevant conflicts of interest to declare.

Imatinib Mesylate Reduces Bone Marrow Fibrosis and Overwhelms the Adverse Prognostic Impact of Reticulin Formation in Patients with Chronic Myeloid Leukemia

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2783-2783
Author(s):  
Eda Tanrikulu Simsek ◽  
Ahmet Emre Eskazan ◽  
Mahir Cengiz ◽  
M. Cem Ar ◽  
Seda Ekizoglu ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Myeloid Leukemia ◽  
Risk Scores ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Significant Difference ◽  
Grade Ii ◽  
Pre Treatment ◽  
Marrow Fibrosis

Abstract Introduction: Imatinib mesylate (IM) is the first tyrosine kinase inhibitor (TKI) licensed for the treatment of chronic myeloid leukemia (CML). Severe bone marrow fibrosis (BMF) has been reported in excess of 40% of the patients with CML at diagnosis. Before TKIs became available, BMF which emerged at diagnosis and/or in the late periods of the disease was defined to be a poor prognostic factor, and it contributed significantly to morbidity and mortality from 10% to 30% in patients with CML. The relationship between BMF and both disease progression and prognosis has been the subject of re-evaluation after the introduction of IM therapy. In patients with CML, it has not been clearly demonstrated yet, whether IM improves the poor prognostic effect of fibrosis, and prevents the new BMF development or not. Aim: The purpose of this study was to evaluate the effects of IM therapy on BMF formation, and the prognostic significance of BMF in patients with CML. Material and Methods: One hundred and thirty-five CML patients were enrolled in the study. Patients' demographics, Sokal risk scores, molecular and cytogenetic responses and follow-up periods were noted from the patients' files retrospectively. All pre- and post-IM bone marrow biopsy samples, which were stained with hematoxylin and eosin, were re-evaluated for the current analysis. Grading of BMF was according to the European consensus decisions, graded as 0-III. The term "last bone marrow biopsy" (LBMB) is referred to a biopsy, which was performed at 18th months or later on during IM treatment. Results: The median age was 44 years (range, 18-92 years), and 78 patients (58%) were male. One hundred and twenty-eight patients (95%) were in chronic phase [CP], 4 patients (3%) were in accelerated phase [AP], and 3 patients (2%) were in blast crisis [BC] at the time of IM initiation. Out of 128 CML-CP patients, one hundred and twenty patients (93%) were in early CP, whereas 8 (7%) were in late CP. The percentage of low, intermediate, and high Sokal risk scores were 35%, 43%, and 22%, respectively. Before IM was initiated, thirty-one patients had received previous treatment modalities (hydroxyurea (HU) in twenty-one, and 10 patients had received interferon plus HU. he median duration of IM treatment was 45 months (range, 2-106 months). The rates of complete hematological response (CHR) at 3rd month, complete cytogenetic response (CCyR) at 12th month, and major molecular response (MMR) at 18th month were 92.4%, 71.6%, and 67.6%, respectively. BMF before the initiation of IM therapy was grade 0 in 52 patients (39%), grade I in 39 patients (29%), grade II in 28 patients (21%), and grade III in 16 patients (12%). There was a positive correlation between the Sokal risk scores and the grades of BMF at diagnosis (r:0.313, p <0.01), and as the Sokal risk scores increase, the grades of BMF also increase and the difference was significant (p =0.025). Also the spleen size significantly differ according to the grade of BMF, and the patients with a higher grade of BMF also had a larger spleen (p <0.001). The control bone marrow biopsies at the 12th month of IM were available in one hundred and four patients, and 70 (67%) of them have grade 0, 26 (25%) of them have grade I, 7 (7%) of them have grade II, and 1 (1%) of them has grade III BMF. After 12 months of IM treatment, the grades of BMF have significantly decreased when compared to the pre-treatment values (p =0.001), and BMF grades in LBMB has shown to be significantly decreased when compared to the pre-treatment values (p <0.001) (Table 1 and Figures 1&2). There was no significant difference regarding the grades of BMF, between the biopsy at 12th months and LBMB (p =0.703). The CCyR rates at 12th month did not differ according to the pre-imatinib BMF grades (p =0.127). There was no significant difference between patients with or without CCyR at 12 months of IM regarding grades of BMF (p =0.785). The MMR rates at 18 months did not differ according to pre-treatment grades of BMF (p =0.112). There was no significantly difference in overall survival rates between initial BMF mild (grade 0-I) and severe (grade II-III) groups (p =0.278). Conclusion: IM can reduce BMF after a long period of follow-up, independently from the molecular and cytogenetic responses. The BMF grades at diagnosis does not have a negative impact on the response to IM treatment. Therefore, the adverse prognostic impact of the marrow fibrosis among CML patients seems to disappear in the era of the TKIs. Disclosures No relevant conflicts of interest to declare.

scholarly journals Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients

2016 ◽  
Vol 91 (9) ◽  
pp. 918-922 ◽  
Author(s):  
Paola Guglielmelli ◽  
Giada Rotunno ◽  
Annalisa Pacilli ◽  
Elisa Rumi ◽  
Vittorio Rosti ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Primary Myelofibrosis ◽  
Prognostic Impact ◽  
Bone Marrow Fibrosis ◽  
Marrow Fibrosis
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