08-14-2010, 10:07 AM
Hematology
**Heparin induced thrombocytopenia
-Occur within 4-10 days of therapy with unfractionated heparin.
-Associated with thrombosis due to platelet activation
-Etio: platelet antibodies that cause limb and life threatening thrombosis at unusual sites including arteries
-Spontaneous bleeding is rare (platelet above 20.000
-RX: immediate stop of a direct thrombin inhibitor (Argatroban “ lepirudin “ Discontinue HEPARIN)
**CTCL: Cutaneous T-Cell Lymphoma / RX by Stages: patch, plaque, tumor, Sezary (systemic)
- Early disease- path or plaque stage ïƒ treated with topical nitrogen mustards “outpatient basis
- Advanced disease: cpmventional radiation therapy or total skin electron beam therapy
- Sesary syndrome stage / systemic disease: systemic chemotherapy “bone marrow transplant
=In Sezary: systemic form of CTCL, after the 2 first phases has persisted for years; where Sezary cells are visible in peripheral smear and entire skin surface is affected by erythematous lesions confused with other pathologies
**Tumor lysis syndrome.- Shortly after chemotherapy with elevations in serum uric acid
ïƒ Progress to urate induced renal failure.
ïƒ POTASIUM is elevated or hyperkalemia due to lysis of neoplastic cells
**POEMS syndrome symptoms associated with osteosclerotic Multiple Myeloma
P: Polyneuropathy - O: Organomegaly - E: Endocrinopathy - M: Multiple myeloma - S: Skin changes
**Sickle cell anemia RX:
1. Symptomatic pain control in acute crisis
2. Increasing oxygen tension to reduce sickling
3. Reduce occlusive crisis through Hydration
4. LONG TERM RX: Hydroxyurea (stimulates production of fetal Hb which will not sickle with low oxygen tension)
** Waldestrom™s Macroglobulinemia
- Low garde malignant lymphoma of plasmacytoid lymphocytes that secrete excesive amounts of IgM. Patient present with hyperviscosity syndrome: Raynaud™s phenomenon, visual disturbances (visual floaters) and protein-protein interaction (platelet dysfunction)
- Dx: Demonstration of IgM spike on plasma Electrophoresis
- BX: Infiltration of bone marrow with plasmacytoid lymphocytes
RX:
-Plasmapheresis to control hyperviscosity initially (because chemo can take several wks)
-Chemotherapy: same as used in MM and alkilating agents and prednisone
Survival: 5 years
**Aspirin: Indicated in pts at risks for strokes or myocardial infarction 2º prevention“analgesic “anti-inflammatory
**Clopidogrel “antiplatelet pts at risk for stroke or MI “pts allergic to ASA
**Warfarin: DVT “ Atrial Fibrillation -artificial heart valves
**Li Fraumeni syndrome: mutation in p53 tumor supressor gene. AD condition and predisposes patient to:
--Sarcomas--CNS tumors --carcinomas.
--HIGH RISK of acquired treatment related Acute Myelogenous Leukemias (t-AML) following treatment with cytotoxic drugs. tAML is universally refractory to known therapies.
**Heparin induced thrombocytopenia
-Occur within 4-10 days of therapy with unfractionated heparin.
-Associated with thrombosis due to platelet activation
-Etio: platelet antibodies that cause limb and life threatening thrombosis at unusual sites including arteries
-Spontaneous bleeding is rare (platelet above 20.000
-RX: immediate stop of a direct thrombin inhibitor (Argatroban “ lepirudin “ Discontinue HEPARIN)
**CTCL: Cutaneous T-Cell Lymphoma / RX by Stages: patch, plaque, tumor, Sezary (systemic)
- Early disease- path or plaque stage ïƒ treated with topical nitrogen mustards “outpatient basis
- Advanced disease: cpmventional radiation therapy or total skin electron beam therapy
- Sesary syndrome stage / systemic disease: systemic chemotherapy “bone marrow transplant
=In Sezary: systemic form of CTCL, after the 2 first phases has persisted for years; where Sezary cells are visible in peripheral smear and entire skin surface is affected by erythematous lesions confused with other pathologies
**Tumor lysis syndrome.- Shortly after chemotherapy with elevations in serum uric acid
ïƒ Progress to urate induced renal failure.
ïƒ POTASIUM is elevated or hyperkalemia due to lysis of neoplastic cells
**POEMS syndrome symptoms associated with osteosclerotic Multiple Myeloma
P: Polyneuropathy - O: Organomegaly - E: Endocrinopathy - M: Multiple myeloma - S: Skin changes
**Sickle cell anemia RX:
1. Symptomatic pain control in acute crisis
2. Increasing oxygen tension to reduce sickling
3. Reduce occlusive crisis through Hydration
4. LONG TERM RX: Hydroxyurea (stimulates production of fetal Hb which will not sickle with low oxygen tension)
** Waldestrom™s Macroglobulinemia
- Low garde malignant lymphoma of plasmacytoid lymphocytes that secrete excesive amounts of IgM. Patient present with hyperviscosity syndrome: Raynaud™s phenomenon, visual disturbances (visual floaters) and protein-protein interaction (platelet dysfunction)
- Dx: Demonstration of IgM spike on plasma Electrophoresis
- BX: Infiltration of bone marrow with plasmacytoid lymphocytes
RX:
-Plasmapheresis to control hyperviscosity initially (because chemo can take several wks)
-Chemotherapy: same as used in MM and alkilating agents and prednisone
Survival: 5 years
**Aspirin: Indicated in pts at risks for strokes or myocardial infarction 2º prevention“analgesic “anti-inflammatory
**Clopidogrel “antiplatelet pts at risk for stroke or MI “pts allergic to ASA
**Warfarin: DVT “ Atrial Fibrillation -artificial heart valves
**Li Fraumeni syndrome: mutation in p53 tumor supressor gene. AD condition and predisposes patient to:
--Sarcomas--CNS tumors --carcinomas.
--HIGH RISK of acquired treatment related Acute Myelogenous Leukemias (t-AML) following treatment with cytotoxic drugs. tAML is universally refractory to known therapies.