This blog is educate those interested in knowing why testing is so important in the diagnosis and treatment of cancer and other blood disorders.
Thursday, March 22, 2012
Myeloproliferative Dimyeloproliferative disorders
Myeloproliferative disease or MPD deals with a group of diseases of the bone marrow in which excess cells are produced.
Myeloproliferative
disorders is the name for a group of conditions that cause blood cells
-- platelets, white blood cells, and red blood cells -- to grow
abnormally in the bone marrow. Though myeloproliferative disorders are
serious, and may pose certain health risks, people with these conditions
often live for many years after diagnosis. The prognosis largely
depends on the type of disorder.
Myeloproliferative disorders include:
Polycythemia
vera -- occurs when the bone marrow produces too many blood cells,
especially red blood cells. More than 95% of people with polycythemia
vera carry the blood mutation JAK2V617F.
Essential
thrombocytosis -- occurs when the body produces too many platelet
cells, which help blood to clot. Clots can block blood vessels leading
to heart attack or stroke.
Primary or
idiopathic myelofibrosis, also known as myelosclerosis -- occurs when
the bone marrow produces too much collagen or fibrous tissue in the bone
marrow. This reduces bone marrow's ability to produce blood cells.
Chronic
myelogenous leukemia (CML) -- cancer of the bone marrow that produces
abnormal granulocytes, a type of white blood cell, in the bone marrow.
Signs and Symptoms:
Many
people with myeloproliferative disorders have no symptoms when their
doctors first make the diagnosis. One symptom shared by all
myeloproliferative disorders, with the exception of essential
thrombocytosis, is an enlarged spleen. An enlarged spleen can cause
abdominal pain and a feeling of fullness.
Many times, especially in the early stages, myeloproliferative
disease does not have symptoms. When it does have signs, they vary from
person to person. If you have symptoms, they may include:
Headache
Fatigue
Shortness of breath
Easy bruising or bleeding
Petechiae (tiny red spots under the skin)
Unexplained weight loss
Night sweats
Fever
Specific disorders in which the bone marrow produces too many cells have similar and yet different symptoms.
Some signs and symptoms of the different types of myeloproliferative disorders include:
Polycythemia vera
Fatigue, general malaise
Trouble breathing
Intense itching after bathing in warm water
Stomachaches
Purple spots or patches on the skin
Nosebleeds, gum or stomach bleeding, or blood in the urine
Throbbing and burning pain in the skin, often with darkened, blotchy areas
Headache and problems with vision
High blood pressure
Blockage of blood vessels. This may cause heart disease, stroke, or gangrene (tissue death) of the arms and legs.
Essential thrombocytosis
Heart attack or stoke
Headache
Burning or throbbing pain, redness, and swelling of the hands and feet
Bruising
Gastrointestinal bleeding or blood in the urine
Primary myelofibrosis
Fatigue, general malaise
Trouble breathing
Anemia
Weight loss
Fever and night sweats
Abnormal bleeding
Chronic myelogenous leukemia (CML)
Fatigue, general malaise
Weight loss or loss of appetite
Fever and night sweats
Bone or joint pain
Heart attack or stroke
Trouble breathing
Gastrointestinal bleeding
Infection
Laboratory Tests
Complete blood count (CBC) and differential CBCs and differentials
are the most frequently ordered tests used to help diagnose and monitor
MPDs. Often ordered as part of a yearly physical exam, they are routine
tests that count the number and relative proportion of each of the
different types of cells in your blood stream. They give your doctor
information about the size, shape, and relative maturity of the blood
cells present in your blood at that moment.
CBCs and differentials can be used to detect WBC, RBC, and platelet
increases, decreases, and abnormalities. They can help determine their
severity, diagnose their cause, monitor the course of a disease, and
monitor the response to treatment.
With polycythemia vera, increased RBCs, platelets, and sometimes
WBCs, may be seen. With myelofibrosis, immature granulocytes and
misshapen immature teardrop-shaped red blood cells are often seen, and
WBC and RBC numbers are often decreased. With thrombocythemia, greatly
increased numbers of platelets are seen along with abnormally large
platelets, platelet clumps, and fragments of megakaryocytes.
Irregularities in cell counts may be due to MPDs, but they may also be due to a variety of other temporary or chronic conditions. Other testing is usually done to confirm or rule out the diagnosis of an MPD. Bone marrow aspiration/biopsyIf a doctor suspects a bone marrow disorder, he may order a bone marrow aspiration or biopsy to collect a small sample of marrow. When a specialist (a pathologist,
oncologist, or hematologist) examines the bone and fluid from the bone
marrow sample under the microscope, he can see the number, size, and
shape of precursor cells (blasts), red and white blood cells, and megakaryocytes (platelet precursors).
He can determine the proportions of mature and immature cells, see any
overgrowth of fibrous tissue, and detect any cancer cells from cancers
that may have spread to the marrow. Most bone marrow disorders can be
diagnosed during this examination.
ABGs (Arterial blood gases)
- This test measures the amount of gases in your arterial blood and may
be done when polycythemia vera is suspected. Low levels of oxygen are
associated with secondary polycythemia. Erythropoietin
is a hormone that stimulates the bone marrow to produce RBCs. With
primary polycythemia, erythropoietin levels will be very low or absent,
but with secondary polycythemia they will be normal or high. Genetic testing is sometimes used in suspected chronic myelogenous leukemia to check for the presence or absence of a Philadelphia (Ph') chromosome or a bcr-abl translocation (see BCR ABL) and in suspected polycythemia vera, myelofibrosis, and essential thrombocythemia for the presence or absence of JAK2 mutations, a gene associated with marrow cell production.
Video of Bone Marrow Biopsy
This video is a lecture about a very common MPD which is polycythemia vera. It is seen frequently in persons who live in high altitudes.
For more information then check the following websites:
http://www.umm.edu/altmed/articles/myeloproliferative-disorders-000114.htm or
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