Autopsy and clinical findings in acute leukemia and chronic myeloproliferative diseases —

Thiele, Jürgen; Laubert, A.; Vykoupil, K. F.; Georgii, A.

doi:10.1016/s0344-0338(85)80142-4

Cited by 13 publications

(5 citation statements)

References 48 publications

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“…In this regard, controversy and discussion persist regarding representativity of BM trephine biopsies as well as subjectivity and inter-observer variability with respect to fiber grading by various experts using different scoring systems [ 55 ]. Previous data suggest that myelofibrotic and osteosclerotic changes reveal a very uniform pattern throughout the whole skeleton [ 56 ] and therefore can be representatively assessed by a BM trephine biopsy. Furthermore, recent data have validated the high reliability of the applied WHO reticulin fibrosis grading system [ 26 ] as well as a strong reproducibility of the used grading for collagen and osteosclerosis [ 25 ].…”

Section: Discussionmentioning

confidence: 99%

Long-term effects of ruxolitinib versus best available therapy on bone marrow fibrosis in patients with myelofibrosis

et al. 2018

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BackgroundMyelofibrosis (MF) is a life-shortening complication of myeloproliferative neoplasms associated with ineffective hematopoiesis, splenomegaly, and progressive bone marrow (BM) fibrosis. The oral Janus kinase (JAK) 1/JAK2 inhibitor ruxolitinib has been shown to improve splenomegaly, symptom burden, and overall survival in patients with intermediate-2 or high-risk MF compared with placebo or best available therapy (BAT).MethodsThe effects of ruxolitinib therapy for up to 66 months on BM morphology in 68 patients with advanced MF with variable BM fibrosis grade were compared with those in 192 matching patients treated with BAT. Available trephine biopsies underwent independent, blinded review by three hematopathologists for consensus-based adjudication of grades for reticulin fibrosis, collagen deposition, and osteosclerosis.ResultsRuxolitinib treatment versus BAT was associated with greater odds of BM fibrosis improvement or stabilization and decreased odds of BM fibrosis worsening based on changes from baseline in reticulin fibrosis grade. Generally, these changes were accompanied by a sustained higher level of individual spleen size reduction and regression of leukoerythroblastosis. Patients with more advanced baseline fibrosis showed lower spleen size response.ConclusionsThe finding that long-term ruxolitinib therapy may reverse or markedly delay BM fibrosis progression in advanced MF suggests that sustained JAK inhibition may be disease-modifying.Trial registrationINCB18424-251, ClinicalTrials.gov identifier NCT00509899.Electronic supplementary materialThe online version of this article (10.1186/s13045-018-0585-5) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Long-term effects of ruxolitinib versus best available therapy on bone marrow fibrosis in patients with myelofibrosis

et al. 2018

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“…The median bone marrow aspirate blast percentage of 64% in Ph+ AML cases was similar to or slightly higher than the 44% median blast percentage reported for AML of all types at presentation. 21 …”

Section: Bone Marrow Morphologic Featuresmentioning

confidence: 99%

Philadelphia Chromosome–Positive Acute Myeloid Leukemia

et al. 2007

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“…Only few studies deal with differences in myelofibrosis in different biopsy sites, which had been done on necropsies. 36,37 Whereas one group observed a uniform pattern of myelosclerotic lesions throughout the skeleton, 36 others postulated myelofibrosis to be a generalized process with heterogeneous grades in different sites of the bone marrow in chronic MPD. 37 This latter finding could help explain the relative instability of myelofibrosis during the disease course, aside from some possible effects of chemotherapy as well.…”

Section: ❚Table 3❚ Evolution Of Different Stages Of Myelofibrosis (Mfmentioning

confidence: 99%

Evolution of Myelofibrosis in Chronic Idiopathic Myelofibrosis as Evidenced in Sequential Bone Marrow Biopsy Specimens

et al. 2003

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Although the new World Health Organization (WHO) classification acknowledges "prefibrotic" phases, progression of myelofibrosis in chronic idiopathic myelofibrosis (cIMF) is controversial because there are only a few studies about sequential biopsy specimens, and they yield conflicting results. The conflicting results might be due to a mixture of different degrees of myelofibrosis and therapy regimens within the respective groups studied. To prove this hypothesis, we studied sequential bone marrow biopsy specimens from patients with cIMF and compared 3 groups with different degrees of myelofibrosis at initial diagnosis with a group of patients with primarily unfibrosed disease who met the WHO criteria for prefibrotic cIMF. Patients receiving chemotherapy were considered separately from patients without treatment. Our results favor a steady progression of myelofibrosis unrelated to therapy modalities, whereas confusing literature data can be explained: fibrosis may remain static or lessen, especially in more advanced stages of cIMF.

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Autopsy and clinical findings in acute leukemia and chronic myeloproliferative diseases —

Cited by 13 publications

References 48 publications

Long-term effects of ruxolitinib versus best available therapy on bone marrow fibrosis in patients with myelofibrosis

Long-term effects of ruxolitinib versus best available therapy on bone marrow fibrosis in patients with myelofibrosis

Philadelphia Chromosome–Positive Acute Myeloid Leukemia

Evolution of Myelofibrosis in Chronic Idiopathic Myelofibrosis as Evidenced in Sequential Bone Marrow Biopsy Specimens

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