Tuesday, October 11, 2016

Brief Discussion On Myeloproliferative Syndromes.

Myeloproliferative Syndromes is the name for a group of conditions that causes blood cells: RBCs, WBCs and platelets to grow abnormally in the bone marrow.

The three major myeloproliferative syndromes are:

1. Polycythemia Vera: It is the most common myleoproliferative disorder and is characterized by an increase in red blood cells (RBC) mass. massive splenomegaly and clinical manifestations related to increased blood viscosity. It also includes neurological manifestations like Vertigo, tinnitus, headache and visual disturbances. Increased RBC mass can also lead to thromboses that can causes complications like myocardial infarction, stroke and peripheral vascular disease.
Polycythemia vera must be distinguished from other causes of increased RBC mass, and this can be done by assaying serum erythropoietin levels. In polycythemia vera serum erythropoietin is low while in other causes  erythropoietin levels are high.
Bone marrow biopsy showing hypercellularity with trilineage growth (panmyelosis) with prominent erythroid, granulocytic, and megakaryocytic proliferation

Management: Patients are effectively managed with phlebotomy. Some patients may require splenectomy, and those with pruritits may benefit from psoralens and UV light.

2. Idiopathic Myelofibrosis: This rare entity is characterized by marrow fibrosis, myeloid metaplasia with extramedullary hematopoiesis and splenomegaly. Evaluation of blood smear reveals tear drop shaped RBC, nucleated RBC and some early granulocytic forms, including promyelocytes.
However many entities may lead to marrow fibrosis and extramedullary hematopoiesis, so the diagnosis of primary idiopathic myelofibrosis is made only when other potential causes are ruled out.
                                                     Fibrotic Bone marrow

Differential Diagnosis:
The following diseases are to be included in differential diagnosis:

  • CML
  • Polycythemia vera
  • Hodgin's disease
  • Cancer metastases to marrow (especially from breast and prostate)
  • Infection ( Particularly granulomatous infections)
  • Hairy cell leukemia
Management: Supportive therapy is usually used. Inhibitors of JAk2 and telomerase have shown activity in reducing splenomegaly and marrow fibrosis in some cases. No study has yet shown a particular drug therapy to improve survival.

3, Essential Thrombocytosis: It is usually noticed incidentally upon routine platelet count done in asymptomatic patients. Many other conditions can also lead to increased platelet count and so the diagnosis is one of exclusion. Platelet count must be > 500,000/microliter, and other known causes of thromocytopenia must be ruled out including CML, iron deficiency anemia, splenectomy, malignancy, infection, hemorrhage, polycythemia vera, myelodysplasia and recovery from vitamin B 12 deficiency. 
Although most patients are asymptomatic some may develop headaches, transient ischemic attacks, or other bleeding or thrombotic disease. 
                                   Hypercellularity with a large number of megakaryocytes

Management: Interferon alpha is an effective thearpy as are anagrelide and hydroxyurea. Treatment should not be given just the absolute platelet count is high in the absence of other symptoms . 


2 comments:

  1. Hello, my name is Betty. i was diagnosed of HIV virus 4 years back. Before then, i was dating this Derrick guy. we had something great going on. Our love life only lasted for 8 months. the sixth and seven month of our relationship, i began feeling very weak every now and then. But i never suspected anything until I kept on experiencing different symptoms like having sore throats, constant headache, body weakness e.t.c. I decided to go for a medical check up. I was tested HIV positive. when the doctor broke the news to me, it was as if my whole world had crumbled. it dawned on me then that Derrick has been lying to me. i was very mad to an extent i almost committed suicide. But there was nothing i could do, other than weep and weep. ever since then, i have been spending heavily on medications, going from one medical clinic or the other so I can look healthy. Though since i was a kid, i love herbs a lot. i take herbal tea with grandma while growing so i am quite familiar with herbs. early last year, i was on the internet and on a particular health blog, to check for herbal medication as alternative. I saw many comments and read many testimonies. Until i came across a particular testimony on how Dr.ugo cured a patient from herpes simplex virus using pure natural herbs. i reached out to the doctor because I also was thinking Herpes had no cure. I told him about my HIV status.He encouraged me not to panic that he will help me get my deliverance. he prepared some herbs and sent them to me. The herbs were in four 70ml bottles and I took them morning and night. i used as he directed, and in less than three weeks, i started having appetite to eat plenty of food and i gained back my strength. the fourth week,I consumed all the herbal meds. He encouraged me to go for a test which i did. This time, the result was negative. I quickly contacted the doctor and informed him of the news. I immediately asked him to prepare for Derrick too. He did and sent it. Today, me and Derrick are HIV free. All thanks goes to God Almighty for healing us through Dr.Ugo. if you are having any kind of sickness at all, Dr.Ugo is the right person to talk to and i assure you, you will be glad you did. His email: ugoherbalhome@gmail.com you can also write him on WhatsApp +2348104990619.

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  2. Nice blog
    Have been totally convinced that HIV,and many others dangerous diseases can be best cured using the Herbal Medicine. Dr.Akpu the great and powerful herbalist cured me with his Herbal remedy. I'm Linda,15 years from Texas. I was born with HIV. I lost parents at my teenager age when I was 4th. I had to grew up with my mum only Sister, whom I loved so much. She was so worried about my life as my condition was her mature problem in life. I had taken lots of antiviral treatment to get ride of the virus not believing what there said HIV has no cure. I was on the internet when i came across blogs of testimony about a lady who where cured of a 7 years diagnose of Herpes by this powerful Herbalist, Dr.Akpu.I wrote down his mail @ Dr.akpupowerherbscenter@gmail.com
    I told my Aunt,luckily it was the same Dr,we where referred to few months back by my pastor in church whom members have being testifying about of his cure We did contacted him and explained my problem to him.He prepared the medicine and sent to me with details of how to use and apply. After a while, I began to feel more strong than never before, I felt so lucky and happy when my Doctor congratulated me that he could found no HIV virus again in me. I knew I was free cos my body was so strong. His herbal medicine should have saved my parents life, i said!
    Thanks so much for reading my article, you should share your testimony if you have cure as well
    Dr.akpupowerherbscenter@gmail.com
    Is the solution

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