Hematology
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| Pathophysiologic Basis for Myeloproliferative Disorders | Acquired clonal abnormalities of the hematopoietic stem cell; May see changes in all stem cell lines (erythroid, myeloid, & pt cells); poss specific chromosomal changes
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| True increase in RBC mass | Primary P. vera
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| Relative increase in RBC mass | Secondary P. vera
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| Conditions causing secondary P. vera | Hypoxia (COPD, heart disease, smoking), renal disease, ACS, dehydration, high altitude, blood doping
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| P. vera: incidence/prevalence | 60 to 70 yrs (mean age at dx: 65 yrs; rare in pt <40); M/F = 1.2 to 1
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| P. vera S/S | Problems related to hyperviscocity & hypervolemia; pruritis (esp after hot showers/baths), thromboses, dyspnea, HA, visual disturbance, tinnitus; LUQ pain, easy bleeding
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| Hallmark of P. vera = | Erythrocytosis
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| P. vera H & H | M: >18.5 ; F: >16.5 (often HCT is > 60%)
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| P. vera mgmt | Therapeutic phlebotomy (goal Hct <45 % M, <43% F). Myelosuppression PRN. Hydroxyurea (watch WBC/PLT). Anagrelide if thrombocytosis. Aspirin 81mg. Antihistamine for pruritis. Allopurinol PRN.
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| P. vera survival prognosis | Median if treated: 11-15 year; untreated = 18 months.
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| Causes of Reactive (Secondary) Thrombocytosis | Severe hemorrhage; splenectomy; neoplasms; chronic inflame dz; post acute infxn; B12 def; meds (vincristine, epi); ETOH
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| Causes of Reactive (Secondary) Thrombocytosis | Severe hemorrhage; splenectomy; neoplasms; chronic inflame dz; post acute infxn; B12 def; meds (vincristine, epi); ETOH
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| Thrombocytosis S/S | Median 50-60 yrs (but all ages); rare in kids; slight female predominance; 1/3 of pts asymptomatic at dx; 2/3 of pts vasomotor s/s (HA, dizziness, visual changes) or complications from thrombosis/bleeding
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| Thrombocytosis findings | Splenomegaly (>25% ); hepatomegaly (20%); leukocytosis, erythrocytosis, mild anemia; occ immature precursor cells and/or large plts; BM bx: increased number of megakaryocytes, else normal
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| Thrombocytosis Mgmt | ASA to prevent thrombosis; Cytoreductive tx (hydroxyurea, anagrelide); Plt pheresis if severe bleed; BM bx for Philadelphia chr.
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| Thrombocytosis Prognosis | 10 y survival = 64-80% ; 1-5% evolve into AML, 10-15% evolve into myelofibrosis
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| Disorder in which bone marrow is replaced with scar tissue, leading to anemia | Myelofibrosis
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| Myelofibrosis findings | Fibrosis on BM; splenomegaly; giant plts; teardrop poikilocytosis
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| Myelofibrosis peak incidence & survival | 50-70 yrs old; median survival is 2-5 yr from onset; occurs in 10 to 30% of pts w/P. vera
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| Cause of Myelofibrosis | Unknown
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| Increased bone marrow production of megakaryocytes leads to increased peripheral platelet count | Myelofibrosis
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| Myelofibrosis S/S | Early: asymptomatic; later: malaise; wt loss; splenomegaly/splenic infarction; hepatomegaly in 50% of pts
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| Myelofibrosis findings | BM aspirate = dry tap; anemia generally increases over time; normochromic-normocytic & mild poik; NRBCs
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| Myelofibrosis mgmt | No tx to reverse/ctrl underlying pathology; tx supportive; mgmt of complications; (Procrit, Aranesp); pRBC & plt txn; Thalidomide & Revlimid? ; allogeneic BM txplt for younger pt? ; Median survival 5 yrs
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| Acquired clonal disorders of the hematopoietic stem cell | MDS
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| ______ cytopenias affect one or more cell lines (RBC, WBC, and/or PLTs) | MDS
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| Dz occurs when blood cells do not develop into mature cells, but rather stay in an immature stage within the BM | MDS
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| Some chromosomal abnormalities (5q – loss of part of the long arm of chromosome 5); “pre-leukemia” | MDS
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| MDS: risk factors include exposure to: | Benzene, radiation, chemotx agents (esp alkylating agents & anthracyclines)
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| MDS pts | Average age ≥ 60 years; pts often asymptomatic; If S/S: fatigue, bleeding, recurrent infxn, fever, splenomegaly, pallor
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| MDS Labs | 85% of pts anemic; 50% neutropenia; 30% thrombocytopenia
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| MDS CBC | Normal or low RBC, WBC, PLTS; Blasts in BM <20%; Pelger-Huet cells (bi-lobed neutrophils)
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| MDS: >20% blasts in BM indicates: | Transition into acute leukemia
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| Blasts in BM <20%; Pelger-Huet cells (bi-lobed neutrophils) seen in: | MDS
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| MDS BM shows: | Hypercellular marrow with delayed/abnormal maturation ( 5q- chromosomal abnormality, ring sideroblasts)
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| MDS Mgmt | Monitor closely (lest transformation); Cytokine and transfusion support; chemotherapy (Thalidomide, Lenolidimide for 5q- syndrome, Azacitadine); Allogeneic BM txplt only curative therapy (most pts too old)
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| MDS: Allogeneic BM txplt may cure ?? % of pts | 30-50%, for pts <60 y.o.
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| Cornerstone for tx of MDS | Supportive care
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| MDS prognosis | Ultimately fatal disease; Infections or bleeding common causes of death
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| Risk of transformation to leukemia depends on: | Percentage of blasts in BM
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| Etiology of multiple myeloma | Etiology unknown. May be associated with pesticides, paper production, leather tanning, exposure to radiation from nukes
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| Replacement of normal bone marrow by plasma cells leads to bone marrow failure | Multiple myeloma
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| Lytic lesions predisposing patients to bone pain, pathologic fractures, and hypercalcemia | Multiple myeloma
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| Multiple myeloma S/S | Fatigue/Anemia; Bone pain (from lytic lesions: back and ribs); Recurrent infxn; Sp cord compression; Unexplained fractures; Kidney failure; Hyperviscosity syndrome
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| Multiple myeloma findings | Anemia; Rouleaux; M-spike on SPEP; Bence-Jones proteins in Urine; Hypercalcemia from bony dz; Renal failure from light chain excretion
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| Multiple myeloma classic triad: | Plasmacytosis (BM bx w/plasma cells > 5%); Bone lytic lesions (on bone survey ); M-protein in serum and/or urine
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| Important to differentiate btw multiple myeloma and: | MGUS (monoclonal gammopathy of unknown significance)
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| Thalidomide may be part of tx for: | Myelofibrosis, MDS, multiple myeloma
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| Multiple myeloma mgmt | Chemo; Local radiation (pain ctrl); Autologous BMT / SCT for LT survival (mortality rate of 40-50%); hypercalcemia tx (bisphosphonates)
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| Multiple myeloma Prognosis: | Median survival w/ transplant = 7 yrs; Median survival with chemo: 3 yrs
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| MGUS prevalence | Present in 1% all adults, 3% over 70yr; Progresses to multiple myeloma 25% of cases
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| What MGUS looks like | Usually, pts have monoclonal IgG spike <2.5g/dL, M-spike remains stable
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| Malignancy of B lymphocytes | Waldenstrom’s Macroglobulinemia
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| Waldenstrom Macroglobulinemia causes overproduction of: | Monoclonal macroglobulin (IgM antibody)
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| Waldenstrom Sx/Sx | Fatigue; hyperviscosity syndrome (nausea, vertigo, visual disturbances, mucosal or GI bleeding); wt loss, HA, cold hypersensitivity, peripheral neuropathy, hepatomegaly, splenomegaly, engorged retinal veins
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| Fatigue, cold hypersensitivity, peripheral neuropathy, engorged retinal veins may indicate: | Waldenstrom Macroglobulinemia
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| Hallmark of Waldenstrom: | Monoclonal IgM spike in SPEP
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| Waldenstrom Macroglobulinemia Findings | Anemia ; Plasmacytic lymphocytes on BM bx; Serum viscosity 1.4 to 1.8 x that of water; Bone radiographs normal
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| Waldenstrom is differentiated from MGUS by: | Presence of bone marrow infiltration
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| Waldenstrom Macroglobulinemia mgmt | If asymptomatic, follow expectantly; Plasmapheresis for hyperviscosity syndrome; Fludarabine & Rituximab prefered to alkylating agent tx; BM txpt? ; Median survival 3-5 yrs
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| Oncologic emergencies | Febrile Neutropenia; SVC Syndrome (superior vena cava syndrome); Tumor Lysis Syndrome; Hypercalcemia; Cord compression (myelomas)
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| Only curative tx for MDS | Allogeneic BMT
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| Type of HL that accounts for 80% of HL cases | Nodular sclerosing HL
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| Giant plts & teardrop poik | Myelofibrosis
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| Lytic lesions | multi myeloma
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| Serum viscosity 1.4-1.8 xH2O visc | Waldenstrom’s
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| Preleukemia | MDS
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| Immunoproferative diseases | Waldenström; Multiple Myeloma; MGUS
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| Anagrelide may be used for: | P vera; thrombocytosis
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| Cytoreductive therapies include: | Anagrelide, hydroxyurea
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| Post-showering pruritis, plethora, dyspnea, HA, visual disturbance, tinnitus, HTN, splenomegaly, engorged retinal veins, thromboses, high H&H | Polycythemia vera
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| 5-20% of P vera cases evolve into this over 20 years | myelofibrosis or acute leukemia
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| "Spent phase" in P vera = | 15% of P vera patients: HSM, anemia, circulating immature WBCs, high WBC. BM: myelofibrosis. Tx supportive. Median survival 2 years. May -> to AML.
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| 3 big causes of secondary erythrocytosis | 1 Reactive 2/2 hypoxia (altitude, pulmo dz, smoking, cyanotic heart dz). 2 Pathologic (renal cell ca, renal dz, uterine fibroid, hepatoma, cerebellar hemangioma, high androgen levels). 3 Relative 2/2 decreased plasma volume (diuretics)
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| Hemochromatosis mgmt | Phlebotomy (goal mild anemia, low ferritin, transferring saturation <30%). +/- chelating agents (dereroxamine IM). Avoid alcohol or vitamin C.
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