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26 Cards in this Set
- Front
- Back
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What is another name for Idiopathic Myelofibrosis?
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Agnogenic Myeloid Metaplasia AMM
AMM = IMF |
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What percentage of leukemias are this type, IMF?
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only 5%
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What is the classic triad that characterizes IMF?
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-Marrow Fibrosis
-Extramedullary Hematopoeisis -Leukoerythroblastosis w/ DACROCYTES |
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why are there dacrocytes in IMF?
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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 |
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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. |
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What occurs in getting the bone marrow aspirate in IMF?
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Dry tap - because of the severe fibrosis!
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What does chromosomal analysis tell you re: IMF?
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Nothing; it's Ph' negative, other chromosomes abnormal but not diagnostic.
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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. |
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which phase of bm fibrosis is treatable?
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the fibrotic phase.
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WAIT! you forgot the third phase of bone marrow fibrosis in IMF:
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Osteosclerotic phase - bone forms.
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What is the body's response to bone formation in the marrow?
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Extramedullary hematopoeisis - so you get severe splenomegaly, and immature cells in PB.
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What is the plt cnt in acute phase IMF? what is the general state of the platelets?
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variable; hypogranular, with decreased functionality.
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What is a particularly characteristic cell abnormality seen in IMF?
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weird megakaryocytes - micromegakaryocytes, mononuclear megs, meg fragments.
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What are cellular functional abnormalities seen in IMF?
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1. Decreased grans
2. Ineffective platelets 3. Anemia |
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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. |
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What is the result of the 3 cellular abnormalities?
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-Can't kill bacteria
-Bleed episodically -Iron defic.anemia |
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What presenting symptom usually prompts the doctor visit in IMF?
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-Splenomegaly, so abdominal discomfort.
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What are 2 symptoms that result from decreased platelets?
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-Petechiae (skin rash)
-GI/urinary tract bleeding |
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what are 4 causes of IMF?
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-Benzene exposure
-Ionizing radiation -P32 treatment for PV -Immunologic metabolism |
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what are the 3 common phases seen in IMF?
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1. Cellular - 10 yrs
2. Anemic 3. Spent |
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What characterizes the cellular phase?
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Panhyperplasia; the marrow still works and isn't boney, so these patient's don't present w/ their splenomegaly.
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how is the anemic phase treated?
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-Transfusions
-Iron supplements -Folic acid |
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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 |
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What special testing is done for IMF?
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bone marrow imaging
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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 |
