Glossary of 4. Idiopathic Myelofibrosis
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- What is another name for Idiopathic Myelofibrosis?
- Agnogenic Myeloid Metaplasia AMM
AMM = IMF
- What percentage of leukemias are this type, IMF?
- only 5%
- What is the classic triad that characterizes IMF?
- -Marrow Fibrosis
-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?
- 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
- What are GRANULOCYTE functional abnormalities seen in IMF?
- -Decreased grans
-Reduced glutathione red. activt
-Impared O2 consumption
-Imparied peroxide production.
- What is the result of the 3 cellular abnormalities?
- -Can't kill bacteria
- 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
-P32 treatment for PV
- what are the 3 common phases seen in IMF?
- 1. Cellular - 10 yrs
- 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?
- 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.
3. Platelet abnormalities
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