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4. Idiopathic Myelofibrosis


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What is another name for Idiopathic Myelofibrosis?
Agnogenic Myeloid Metaplasia AMM

What percentage of leukemias are this type, IMF?
only 5%
What is the classic triad that characterizes IMF?
-Marrow Fibrosis
-Extramedullary Hematopoeisis
-Leukoerythroblastosis w/ DACROCYTES
why are there dacrocytes in IMF?
because as the cells go through little places, the sinusoids are filled up w/ fibrosis and it stretches RBCs emerging from marrow severly.
what sort of WBC cnt is in IMF?
Anemia type?
Variable, 10000-40000; unsevere.
What sort of diff do you see in IMF?
-Granulocytes at all different developmental stages
-plenty of nRBCs, monos, basos, and eos.
What occurs in getting the bone marrow aspirate in IMF?
Dry tap - because of the severe fibrosis!
What does chromosomal analysis tell you re: IMF?
Nothing; it's Ph' negative, other chromosomes abnormal but not diagnostic.
what are two possible phases regarding the bone marrow in acute phase IMF?
-Fibrotic phase, sees mostly reticular fibrosis

-Sclerotic phase, sees collagenic fibrosis.
which phase of bm fibrosis is treatable?
the fibrotic phase.
WAIT! you forgot the third phase of bone marrow fibrosis in IMF:
Osteosclerotic phase - bone forms.
What is the body's response to bone formation in the marrow?
Extramedullary hematopoeisis - so you get severe splenomegaly, and immature cells in PB.
What is the plt cnt in acute phase IMF? what is the general state of the platelets?
variable; hypogranular, with decreased functionality.
What is a particularly characteristic cell abnormality seen in IMF?
weird megakaryocytes - micromegakaryocytes, mononuclear megs, meg fragments.
What are cellular functional abnormalities seen in IMF?
1. Decreased grans
2. Ineffective platelets
3. Anemia
What are GRANULOCYTE functional abnormalities seen in IMF?
-Decreased grans
-Myeloperoxidase defic.
-Reduced glutathione red. activt
-Impaired phagocytosis
-Impared O2 consumption
-Imparied peroxide production.
What is the result of the 3 cellular abnormalities?
-Can't kill bacteria
-Bleed episodically
-Iron defic.anemia
What presenting symptom usually prompts the doctor visit in IMF?
-Splenomegaly, so abdominal discomfort.
What are 2 symptoms that result from decreased platelets?
-Petechiae (skin rash)
-GI/urinary tract bleeding
what are 4 causes of IMF?
-Benzene exposure
-Ionizing radiation
-P32 treatment for PV
-Immunologic metabolism
what are the 3 common phases seen in IMF?
1. Cellular - 10 yrs
2. Anemic
3. Spent
What characterizes the cellular phase?
Panhyperplasia; the marrow still works and isn't boney, so these patient's don't present w/ their splenomegaly.
how is the anemic phase treated?
-Iron supplements
-Folic acid
For what 3 reasons would you splenectomize an IMF patient?
why would you not?
1. Spleen painfully enlarged
2. SEvere hemolytic anemia
3. Severe thrombocytopenia
-Reduces extramed hematopoeises
What special testing is done for IMF?
bone marrow imaging
What are 3 main causes for symptoms seen in IMF?
1. Organomegaly - from Extra-medullary hematopoeisis.
2. Anemia
3. Platelet abnormalities

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