Case of the day

A Rare case of Myelofibrosis by Dr. Vinod Shah

Myelofibrosis is a rare type of blood cancer (chronic leukemia) which is characterized by replacement of bone marrow with fibrous scar tissue. Share your views on this rare case by Dr. Vinod Shah & learn through his rich experience! Follow us for more such updates!

66 Likes

LikeAnswersShare

A Rare case of Myelofibrosis very nicely presented by Dr Vinod Shah in Experts Insights case .As such cases may present with very vague presentations- bone marrow biopsy remains the mainstay of treatment Pathophysiology In patients with primary myelofibrosis, the hematopoietic system is most affected. Other organ systems may be involved via extramedullary hematopoiesis. Clonality studies in patients with primary myelofibrosis demonstrate that myeloid cells arise from clonal stem cells; however, bone marrow fibroblasts and, sometimes, T cells are polyclonal. The cause of the excessive marrow fibrosis observed in primary myelofibrosis remains unclear. Platelets, megakaryocytes, and monocytes are thought to secrete several cytokines, such as transforming growth factor beta (TGF-β), platelet-derived growth factor (PDGF), interleukin 1 (IL-1), epidermal growth factor (EGF), and basic fibroblast growth factor (bFGF), which may result in fibroblast formation and extracellular matrix proliferation. In addition, endothelial proliferation and growth of capillary blood vessels in the bone marrow are observed and may be a result of TGF-β and bFGF production. Neoangiogenesis is a hallmark feature of chronic myeloproliferative disorders. Approximately 70% of patients with primary myelofibrosis have substantial increases in bone marrow microvessel density. Neoangiogenesis in primary myelofibrosis is noted in medullary and extramedullary hematopoiesis. Increased serum vascular endothelial growth factor levels have been postulated as the underlying mechanism for increased angiogenesis.

NICE ILLUSTRATION MYELOFIBROSIS IS A KIND OF DISIRD CHARETERISED BY SCARING NOF BONE MARROW.LEADIND TO SEVERE ANEAMIA EASY FATGIBILITY LOW PLATELET COUNT PTS PRESENT WITH BRUISING EASY BLEEDING FATGIBILITY BONE PAIN CAUSES AGE EXPOSURE TO CHEMICAL EXPISURE TO RADIATIONS

I AGREE WITH DR.GOWHAR AHMAD.THANKS
0

Congratulations! Your case has been selected as Case of the day and you have been awarded 5 points for sharing the case. Keep posting your interesting cases, Happy Curofying!

No blood is formed so repeated Blood transfusion required.. Gradually bone marrow may become functional and person may live normal life. Otherwise Bone marrow transplant is only tt.

At present ,since last 2 months of her high tc and pc her hb has not gone bellow 10.2 . So yet no BT is given It was before25 yrs back during her 2 nd pregnancy BT was once given. Old reports r not available.
0

View 3 other replies

My interest to put this case in Curofy is that most of the members r General Practitioner and would know that high tc and pc should investigated thoroughly.

So this patient has essential thrombocythemia and not myelofibrosis. Additionally, JAK 16F mutations are a high risk for thrombosis, and the patient should also be put on Aspirin BID along with hydrea (aim platelet count <4,50,000, so increase the dose of hydrea as tolerated). Also prior to starting aspirin with that high platelet count you should rule out acquired Von Willebrand disease.

As sir,only 9 days has been passed after starting the medicine. Every 15 days the count of Tc andPc will b checked . Close watching is kept on pt for any untowards complain. Will update regularly.
1

Rare disease . Informative and educative post. Repeated BT. Thanks for sharing.

Thanks Dr Anil Gangani.
0

View 2 other replies

Excellent not heard of My brother CML Surviving last 16 yr on Drugs help by max foundation Free

Can u send me the contact no of max foundation so my pt can get treatment at low cost or free??
1

View 3 other replies

An important and helpful educative post, thanks@Expert Insights and also thank you Sir@Dr. Vinod Shah .

Thank you Sir@Dr. Ashok Leel .
0

Beetroot sweet potato... ginger carrots little garlic...lime juice... pineapple with black pepper... sproutskalijeeri drumstick soup flaxseed.. cauliflower broccoli purple cabbage spinach.. chiaseeds help..

Thanks ji Doctor Saheb
0
Load more answers

Cases that would interest you