In addition to improvements in spleen volume and constitutional symptoms, the interim data suggest improvements in anemia, transfusion dependence, and
bone marrow fibrosis.
These two patients have remained transfusion independent for more than 69 and 24 weeks, respectively, as of April 17 and remain on trial.mTen patients were evaluable for
bone marrow fibrosis, of which six experienced improvement in bone marrow morphology of at least one point on a scale of 0-3.
Thrombocytopenia with mild
bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly.
Assessments include measuring changes in spleen volume, patient reported total symptom scores, mutant allele burden, inflammatory cytokines as well as
bone marrow fibrosis over the course of the treatment period.
These result in the secondary process of
bone marrow fibrosis.
Bone marrow fibrosis which impairs the normal haematopoietic process causing severe anaemia is the hallmark of Primary Myelofibrosis.4
For
bone marrow fibrosis, the difference was not statistically significant (p>0.05).
Al-Ibrahimi conducted successful bone marrow transplants on 12 Bahraini children in Jordan over the past years -- 2 cases of leukemia, 5 cases of Thalassemia, 1 case of bone marrow failure syndrome, 1 case of
Bone marrow fibrosis, 1 case of Gideak Hakashy Syndrome and one case of Autologous bone marrow transplant.
The possible mechanism of PHPT causing anemia and marrow fibrosis is that PTH has a stimulatory effect on marrow fibroblasts, leading to
bone marrow fibrosis, but also to the release of cytokines IL-6 and TNF-[alpha] from the osteoclasts that play an indirect role in the pathogenesis of fibrosis [7, 8, 18].
Subtle focal intrasinusoidal collections of the cells, active bone remodelling and
bone marrow fibrosis were also noted in these subcortical areas.
European consensus on grading
bone marrow fibrosis and assessment of cellularity.
Objective:
Bone marrow fibrosis is the second most common complication that causes morbidity and mortality in patients with Philadelphia-negative myeloproliferative neoplasms (MPNs).
In summary, the MPNs are involved with one or more of the following features: overproduction of one or more cell lines, hypercellular marrow or
bone marrow fibrosis, cytogenetic abnormalities, point mutations, extramedullary hematopoiesis, hemorrhagic bleeding and or thrombosis and can transform into an acute leukemia.