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37 Cards in this Set
- Front
- Back
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MYELOPROLIFERATIVE DISORDERS (MPD)
- define |
- Acquired CLONAL disorder
characterized by Expansion of 1 or more hematopoietic stem cell compartments |
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MYELOPROLIFERATIVE DISORDERS (MPD)
- MPD is not only Clonal, but also ____ and escape what? |
- neoplastic
- normal cellular growth regulations |
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MYELOPROLIFERATIVE DISORDERS (MPD)
- MPD's are prone to developing what? |
- Acute Leukemia
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MYELOPROLIFERATIVE DISORDERS (MPD)
- 4 categories? |
- Polycythemia Vera
- CML - Essential Thrombocytosis - Idiopathic Myelofibrosis |
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MYELOPROLIFERATIVE DISORDERS (MPD)
Associations with Excessive proliferation in: - Polycythemia Vera (PV)? - CML? - Essential Thrombocytosis? - Idiopathic Myelofibrosis |
- Erythroid, Myeloid, Megakaryocyctic
- Myeloid, Megakaryocytic - Megakaryocytic - Fibrosis + all 3 |
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POLYCYTHEMIA VERA (PV)
- Hypoxemia is seen in Primary or Secondary PV? |
- Secondary PV
(hypoxemia people make lots of RBCs to compensate, so they have Polycythemia, but not PV) |
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POLYCYTHEMIA VERA (PV)
- symptoms x8 |
- Fullness in abdomen
- Itching - Tinnitis - HA - Dizzy - Blurry vision - Hemorrhage - Thrombosis |
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POLYCYTHEMIA VERA (PV)
- good to note what on PE? - shows what? x2 |
- Face
- Plethoric (redness of face) - Rosacea (pimples) |
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POLYCYTHEMIA VERA (PV)
- patients with PV sometimes come into to MD with what on their joints? |
- gout
- cell proliferation causes increased cell destruction leading to excess purine and pyrimidine destruction. One of the byproducts is Uric acid, which can lead to gouty tophus |
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POLYCYTHEMIA VERA (PV)
- Secondary Polycythemia and those with Cyanotic CHD may present to MD with what on their hands? |
- Clubbing
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POLYCYTHEMIA VERA (PV)
- likely age of diagnosis? - ratio of men to women? |
- 67.1
- 1.2:1 |
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POLYCYTHEMIA VERA (PV)
- discuss hematocrit in PV - discuss hematocrit in Secondary Polycythemia |
- Increased
- may be elevated (higher RC mass), but also be due to dehydration (lower plasma volume). So be careful with this test and look at the relativity |
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POLYCYTHEMIA VERA (PV)
- PV or Secondary Polycythemia must have elevated what? |
- RCM (Red cell mass)
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POLYCYTHEMIA VERA (PV)
- causes of Secondary Polycythemia x3 |
- Hypoxia
(hypoxic lung dz, high altitude, cyanotic CHD, smoking, abnormal Hb) - Inappropriate secretion of EPO (PURCH - pheochromocytoma, uterine fibroids, renal dz, cerebellar hemangioblastoma, hepatoma) - other (androgens, neonatal polycythemia, hypertransfusion) |
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POLYCYTHEMIA VERA (PV)
- Diagnostic Category A (x3) |
Total RCM
- Males: >= 36 ml/kg body wt. - Females >= 32 ml/kg body wt. Splenomegaly Arterial O2 sat > 92% |
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POLYCYTHEMIA VERA (PV)
- Diagnostic category B (x4) |
(SALT)
- Serum vitamin B12 >900 pg/mL (or B12 binding > 2200 pg/mL) - Alkaline Phosphate score increased - Leukocytosis (WBC > 12,000 / mm3) - Thrombocytosis (PLT > 4,000,000) |
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POLYCYTHEMIA VERA (PV)
- EPO used how diagnostically for PV? |
- High EPO indicates Secondary Polycythemia
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POLYCYTHEMIA VERA (PV)
- If EPO is low, then what followup test? - results indicating PV? |
- Bone Marrow Biopsy with histo
- 95% cellularity = PV (normal is 50% with rest fat) |
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POLYCYTHEMIA VERA (PV)
- genetic association? - what else has same genetic association? |
- JAK2
(tyrosine Kinase JAnus kinase2 gene) (all except CML) |
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POLYCYTHEMIA VERA (PV)
- Tx in patients with Thrombosis? - Tx in pts without Thrombocytosis and previous Hx of thrombosis? - Tx in pts with Persistent Thrombocytosis - Elderly pts and Non-responsives |
- Phlebotomy
- Phlebotomy with Hydroxyurea - Anti-platelet (Anagrelide or Interferon-alpha) - (32)P 2.3 mCi/m2 IV every 12 wks (to suppress thrombocytosis) |
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CML
- expansion of what in bone marrow? - expansion of what in peripheral blood? |
- myeloid and megakaryocyctic
- myeloid and megakaryocytic |
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CML
- genetic association? |
- ABL:BCR t(9:22)
(philly chr) |
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CML
- Tx x4 |
- Gleevac
- IFN & AraC - Hydroxyurea - Allogeneic bone marrow transplant |
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ESSENTIAL THROMBOCYTOSIS (ET)
- characterized by increased? x2 |
- platelets
- Megakaryocytes |
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ESSENTIAL THROMBOCYTOSIS (ET)
- bone marrow shows what? |
- clusters (islands) of megakaryocytes
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ESSENTIAL THROMBOCYTOSIS (ET)
RISK STRATIFICATION - High risk? x2 |
- Age >= 60
- Previous Hx of Thrombosis |
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ESSENTIAL THROMBOCYTOSIS (ET)
RISK STRATIFICATION - Low risk? x4 |
- Age < 60
- No Hx of Thrombosis - Platelet < 1.5 million / uL - No CV risk factors |
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ESSENTIAL THROMBOCYTOSIS (ET)
TREATMENT - for Low risk? |
nothing
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ESSENTIAL THROMBOCYTOSIS (ET)
TREATMENT - for intermediate risk? |
- No treatment
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ESSENTIAL THROMBOCYTOSIS (ET)
TREATMENT - for high risk? |
If < 60
- Hydroxyurea (or Anagrelide) If >= 60 - Hydroxyurea If pregnant - IFN-alpha |
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ESSENTIAL THROMBOCYTOSIS (ET)
TREATMENT - in what group would ASA be contraindicated? |
- Intermediate risk UNDER 60
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MYELOFIBROSIS
- main characters? x2 |
- Excessive Collagen Disposition in BM
- Extramedullary Hematopoiesis |
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MYELOFIBROSIS
- very common finding in >50% cases? x6 |
(T-FLASH)
- Thrombocytosis - Fatigue - Leukocytosis - Anemia - Splenomegaly - Hepatomegaly |
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MYELOFIBROSIS
- major PE finding? |
- Massive Splenomegaly
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MYELOFIBROSIS
- Histo finding in BM? - Peripheral blood smear finding? |
- Fibrosis
- Osteosclerosis - Reticular fibers (silver staining) - Teardrop cell (myeloid metaplasia & myelofibrosis) |
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MYELOFIBROSIS
- Treatment? - Recent advanced findings on 2 drugs? - what about those with poor prognosis? |
Symptomatic
- Thalidomide - Lenolidomide - BM transplants |
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CML
- Tx? x3 |
- Gleevac
- Interferon ALPHA - BM transplants |
