4. Idiopathic Myelofibrosis
Terms
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- What is another name for Idiopathic Myelofibrosis?
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Agnogenic Myeloid Metaplasia AMM
AMM = IMF - What percentage of leukemias are this type, IMF?
- only 5%
- What is the classic triad that characterizes IMF?
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-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.
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what sort of WBC cnt is in IMF?
Anemia type? -
Variable, 10000-40000; unsevere.
Normo/normo - What sort of diff do you see in IMF?
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-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?
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-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?
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1. Decreased grans
2. Ineffective platelets
3. Anemia - What are GRANULOCYTE functional abnormalities seen in IMF?
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-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?
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-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?
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-Petechiae (skin rash)
-GI/urinary tract bleeding - what are 4 causes of IMF?
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-Benzene exposure
-Ionizing radiation
-P32 treatment for PV
-Immunologic metabolism - what are the 3 common phases seen in IMF?
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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?
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-Transfusions
-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?
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1. Organomegaly - from Extra-medullary hematopoeisis.
2. Anemia
3. Platelet abnormalities