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Hemo Malignancies


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
True increase in RBC mass Primary P. vera
Relative increase in RBC mass Secondary P. vera
Conditions causing secondary P. vera Conditions such as hypoxia (COPD, heart disease, cigarette smoking), renal disease, MI’s, dehydration, high altitude, autotransfusion (blood doping
P. vera: incidence/prevalence 60 to 70 yrs (mean age at dx: 65 yrs; rare in pt <40); M/F = 1.2 to 1
P. vera S/S Probs rel to hyperviscocity & hypervolemia; pruritis (esp. after hot showers/baths), thromboses; dyspnea; HA; visual disturbance; tinnitus
Hallmark of P. vera = Erythrocytosis
P. vera H & H M: >18.5 ; F: >16.5 (often HCT is > 60%)
Condition w/thrombosis; splenomegaly; Plethora; systolic hypertension; engorged retinal veins P. vera
P. vera lab data Positive JAK-2 (janus kinase) serum test (pos in 74-97% of pts)
P. vera mgmt Tx phlebotomies (Hct <45 % M; Hct <43% F); myelosuppression if nec; hydroxyurea (watch WBC/PLT); Anagrelide if thrombocytosis; Aspirin 81mg to reduce thrombosis risk; antihistamine for pruritis
P. vera mgmt Tx phlebotomies (Hct <45 % M; Hct <43% F); myelosuppression if nec; hydroxyurea (watch WBC/PLT); Anagrelide if thrombocytosis; Aspirin 81mg to reduce thrombosis risk; antihistamine for pruritis
P. vera prognosis Median Survival Tx: 11-15 yr; untx’d = 18 mos; 5-20% evolve into myelofibrosis or acute leukemia over 20 yrs
Causes of Reactive (Secondary) Thrombocytosis Severe hemorrhage; splenectomy; neoplasms; chronic inflame dz; post acute infxn; B12 def; meds (vincristine, epi); ETOH
Causes of Reactive (Secondary) Thrombocytosis Severe hemorrhage; splenectomy; neoplasms; chronic inflame dz; post acute infxn; B12 def; meds (vincristine, epi); ETOH
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
Thrombocytosis findings Splenomegaly (25+% ); hepatomegaly (20 %); Leukocytosis, erythrocytosis, mild anemia; occ immature precursor cells and/or large plts; BM bx: inc no. of megakaryocytes, else normal
Thrombocytosis Mgmt ASA to prevent thrombosis; Cytoreductive tx (hydroxyurea, anagrelide); Plt pheresis if severe bleed; BM bx for Philadelphia chr.
Thrombocytosis Prognosis 10 y survival = 64-80% ; 1-5% evolve into AML, 10-15% evolve into myelofibrosis
Disorder in which bone marrow is replaced with scar tissue, leading to anemia Myelofibrosis
Myelofibrosis findings Fibrosis on BM; splenomegaly; giant plts; teardrop poikilocytosis
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
Cause of Myelofibrosis Unknown
JAK-2 mutations may be associated with: Myelofibrosis, thrombocytosis; P vera = often pos JAK-2 serum test
Increased bone marrow production of megakaryocytes leads to increased peripheral platelet count Myelofibrosis
Myelofibrosis S/S Early: asymptomatic; later: malaise; wt loss; splenomegaly/splenic infarction; hepatomegaly in 50% of pts
Myelofibrosis findings BM aspirate = dry tap; anemia generally increases over time; normochromic-normocytic & mild poik; NRBCs
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
Acquired clonal disorders of the hematopoietic stem cell MDS
______ cytopenias affect one or more cell lines (RBC, WBC, and/or PLTs) MDS
Dz occurs when blood cells do not develop into mature cells, but rather stay in an immature stage within the BM MDS
Some chromosomal abnormalities (5q – loss of part of the long arm of chromosome 5); “pre-leukemia” MDS
MDS: risk factors include exposure to: Benzene, radiation, chemotx agents (esp alkylating agents & anthracyclines)
MDS pts Average age ≥ 60 years; pts often asymptomatic; If S/S: fatigue, bleeding, recurrent infxn, fever, splenomegaly, pallor
MDS Labs 85% of pts anemic; 50% neutropenia; 30% thrombocytopenia
MDS CBC Normal or low RBC, WBC, PLTS; Blasts in BM <20%; Pelger-Huet cells (bi-lobed neutrophils)
MDS: >20% blasts in BM indicates: Transition into acute leukemia
Blasts in BM <20%; Pelger-Huet cells (bi-lobed neutrophils) seen in: MDS
MDS BM shows: Hypercellular marrow with delayed/abnormal maturation ( 5q- chromosomal abnormality, ring sideroblasts)
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)
MDS: Allogeneic BM txplt may cure ?? % of pts 30-50%, for pts <60 y.o.
Cornerstone for tx of MDS Supportive care
MDS prognosis Ultimately fatal disease; Infections or bleeding common causes of death
Risk of transformation to leukemia depends on: Percentage of blasts in BM
Etiology of multiple myeloma; assoc with: Etiology unknown; assoc w/pesticides, paper production, leather tanning, exposure to radiation from nukes
Replacement of normal bone marrow by plasma cells leads to bone marrow failure Multiple myeloma
Lytic lesions predisposing patients to bone pain, pathologic fractures, and hypercalcemia Multiple myeloma
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
Multiple myeloma findings Anemia; Rouleaux; M-spike on SPEP; Bence-Jones proteins in Urine; Hypercalcemia from bony dz; Renal failure from light chain excretion
Multiple myeloma classic triad: Plasmacytosis (BM bx w/plasma cells > 5%); Bone lytic lesions (on bone survey ); M-protein in serum and/or urine
Important to differentiate btw multiple myeloma and: MGUS (monoclonal gammopathy of unknown significance)
Multiple myeloma evaluation SPEP (M-spike); 24 hr urine (Bence-Jones pro); Serum viscosity (may req plasma pheresis); Metastatic bone survey (lytic lesions); BM bx (inc. cytogenetics for q13- chromosome abnormality); Beta-2 microglobulins
Part of eval for multiple myeloma (if elevated = very poor outcome) Beta-2 microglobulins
Thalidomide may be part of tx for: Myelofibrosis, MDS, multiple myeloma
Multiple myeloma mgmt Chemo; Local radiation (pain ctrl); Autologous BM txplt for LT survival (mortality rate of 40-50%); hypercalcemia tx (bisphosphonates)
Multiple myeloma Prognosis: Median survival w/ transplant = 7 yrs; Median survival with chemo: 3 yrs
MGUS prevalence Present in 1% all adults, 3% over 70yr; Progresses to multiple myeloma 25% of cases
What MGUS looks like Usually, pts have monoclonal IgG spike <2.5g/dL, M-spike remains stable
Malignancy of B lymphocytes Waldenstrom’s Macroglobulinemia
Waldenstrom’s Macroglobulinemia causes overproduction of: Monoclonal macroglobulin (IgM antibody)
Median age of onset is 64 yrs Waldenstrom’s
Waldenstrom’s S/S Present w/fatigue; Hyperviscosity syndrome (nausea, vertigo, visual disturbances, mucosal or GI bleeding); wt loss, HA, cold hypersensitivity, peripheral neuropathy, hepatomegaly, splenomegaly, engorged retinal veins
Fatigue, cold hypersensitivity, peripheral neuropathy, engorged retinal veins may indicate: Waldenstrom’s Macroglobulinemia
Hallmark of Waldenstrom: Monoclonal IgM spike in SPEP
Waldenstrom’s Macroglobulinemia Findings Anemia ; Plasmacytic lymphocytes on BM bx; Serum viscosity 1.4 to 1.8 x that of water; Bone radiographs normal
Waldenstrom’s is differentiated from MGUS by: Presence of bone marrow infiltration
Waldenstrom’s Macroglobulinemia mgmt If asymptomatic, follow expectantly; Plasmapheresis for hyperviscosity syndrome; Fludarabine & Rituximab prefered to alkylating agent tx; BM txpt? ; Median survival 3-5 yrs
Most commonly occurring leukemia CLL
CLL Mgmt Observation; chemo; BMT; Tumor lysis prophylaxis (allopurinol, 300 mg/day, hydration, diuretics); Radiation (ctrl bulky adenopathy); Surgery (for diagnostic)
CLL prevalence Mainly disease of older people (>90% of cases >50yr); M:F = 2:1
Characterized by clonal proliferation and accumulation of mature-appearing B lymphocytes (95 % of cases) in blood and lymphoid tissue; clonal malignancy of B lymphs CLL
CLL S/S 25 % asymptomatic at dx; fatigue; drenching night sweats; lymphadenopathy (80%); wt loss; frequent/persistent infxn; skin infxn/shingles
CLL findings Anemia & thrombocytopenia; mature small lymphs & smudge cells; Hepatomegaly; Splenomegaly
RAI Staging System used for: CLL
CLL growth Tends to be slow growing and indolent
Lymphocytosis, WBCs >20,000/µL = hallmark of: CLL
RAI Stage 0 Inc WBC (leukocytosis blood & marrow); >150 mos survival
RAI Stage 1 Inc WBC & lymphadenopathy; >101 mos survival
RAI Stage 2 Inc WBC & Hepato/splenomegaly; >71 mos survival
RAI Stage 3 Inc WBC & Anemia (Hgb < 11g/dl); >19 mos survival
RAI Stage 4 Inc WBC & Thrombocytopenia; >19 mos survival
Blast crisis When blasts comprise >30% of BM cells
CML will likely transform to: Acute disease
Primarily disease of children ALL
?? % of ALL pts are children (usually 3 -7 yo) 80%
Accounts for 10-20% of acute adult leukemia ALL
CML mgmt Allo BMT; chemo (Gleevec; Dasatinib, AMN107; Interferon)
3 phases of CML (defined by # blasts in marrow) Chronic, accelerated, and acute
Slowly progressing dz: too many WBCs made in BM (esp myeloid cells) CML
CML prevalence Usually in young to middle age adults (medium age: 42); CML accounts for 7-20% cases of leukemia
Philadelphia chromosome present in ??% of CML cases >95% of cases (t9:22 translocation of DNA)
_____ can trigger Philadelphia chromosome Exposure to radiation
CML S/S Fever (w/o infxn); bone pain; LUQ pain (enlarged spleen); night sweats; bleeding & bruising; petechiae; fatigue ; weakness
CML findings Median WBC = 170,000 at dx; splenomegaly; BM/PBS: Phil. Chr (bcr/abl fusion gene); FISH for bcr/abl (quantitative); RT-PCR for bcr/abl (qualitative); thrombocytopenia (30%)
CML tx goal: Complete hemo & cytogenetic response; Five yr survival = 52-63% ; Median survival =6 yrs
Type of leukemia = immature, abnormal cells in BM (>20%) and blood (>10%) & in liver, spleen, lymph nodes Acute Leukemia
ALL prognosis 80% of children will be cured with chemo; 20-40% of adults will be cured
ALL & AML S/S The usual S/S plus meningitis; Fever (abrupt onset with children); petechiae; anorexia
ALL & AML labs Pancytopenia; hyperleukocytosis; BM >20% blasts
AML: median age at onset: 65 yr
AML: 5 year survival: 10-30%
Hyperleukocytosis Circulating blasts in peripheral blood (> 200,000/ mcl)
ALL mgmt Aggressive combo chemo [approx 2 yrs total (cytoxan, donorubicin, vincristine, prednisone)]; CNS prophylaxis (intrathecal chemo); BMT??
Chemo phases for ALL Induction phase (4-6 wks); Consolidation phase (several mos); Maintenance phase (2-3 yrs)
AML Etiology Poss: exposure to toxins (benzenes, radiation, chemotherapy)
AML findings Pancytopenia (& circulating blasts) (poss leukocytosis > 200,000); BM >20% blasts; Auer Rods; high ESR; hepatosplenomegaly
AML mgmt Chemo; Induction & 3 consolidation treatments, in hospital; Chemo = ARA-C with mitoxantrone, idarubicin, or daunorubicin; BMT; Tumor lysis prophylaxis (allopurinol, 300 mg/day, hydration, diuretics)
Hairy Cell leukemia prevalence Median age of onset = mid 50s; M:F = 5:1; usually indolent, very responsive to tx
Hairy Cell leukemia presentation Fatigue, abd discomfort (markedly enlarged spleen); persistent infxn;
Hairy Cell leukemia tx 2-CDA (2-chlorodeoxyadenosine), oral for 5-7 days (watch for drop in CBC counts); 90% of pts complete remission
Dz’s tx’d w/tumor lysis AML &CLL & NHL
Hallmark of Hairy Cell leukemia Pancytopenia; “hairy” cells
Hodgkin Dz Nodular Sclerosing (80%); LPHD; Classical Hodgkin Dz; Mixed cellularity
Non-Hodgkin Dz Follicular; Burkitt; Diffuse lg B-cell; Marginal zone; Cutaneous T-cell; Anaplastic large cell
Group of cancers w/enlarged lymphoid tissue, spleen, liver, & Reed-Sternberg cells Hodgkin Dz
Hodgkin Dz prevalence 7,900 new cases annually, two peaks for age of onset: age 20-40 then after 50; rare in kids <5; more common in men 15-45
_____ found in 40 – 50% of Hodgkin dz cases EBV
Reed-Sternberg cells Hodgkin Dz
Hodgkin Dz mechm of spread in pt Usually arises in a single area and spreads to contiguous nodes (“next-door dz”)
Hodgkin Dz S/S Painless cervical , supraclavicular, & mediastinal lymphadenopathy; constitutional B symptoms (fever, drenching night sweats, wt loss); SOB common with mediastinal mass
Hodgkin Dz findings Nodular sclerosis (esp young pt); Mixed cellularity (esp in older pts); prob infxs etiology in young pts (mononuc, x3 risk); 5-10% = extranodal presentation (lung, liver, bone marrow); Chest Xray: often mediastinal mass
Hodgkin Dz diagnostics CT chest, abdomen, pelvis; PET scan; BM bx
Hodgkin Dz Stage I: one lymph node region
Hodgkin Dz Stage II: two lymph node areas but only one side of the diaphragm
Hodgkin Dz Stage III: nodal disease, both above and below the diaphragm
Hodgkin Dz Stage IV: extranodal disease
Hodgkin Dz mgmt: Stage I and IIa: local disease, radiation alone
Hodgkin Dz mgmt: Stage IIb: Controversial, radiation +/- chemotherapy
Hodgkin Dz mgmt: Stage III: nodal disease, both above and below the diaphragm, chemo +/-radiation (ABVD)
Hodgkin Dz mgmt: Stage IV: Extranodal disease, chemotherapy
Hodgkin Dz: Five year survival rate: > 80% (Will usually recur in 2 yrs if at all)
Hodgkin Dz: if relapse: Consider high dose chemo followed by BMT
Hodgkin Dz mgmt Chemo: ABVD; give chemo q 2 weeks x 4-6 cycles; Re-scan after 2-3 cycles to detn response; Radiation follows chemotherapy; Watch for toxicity/neutropenia (use CSFs); sperm banking?
5th most common malignancy in US NHL
NHL prevalence Approx. 50,000 new cases annually; incidence higher in pts w/immunodeficiencies, h/o EBV, exposures to pesticides/solvents
Dz: arise from cells in Lymphoid tissue (90% of cases are derived from B-lympocytes) NHL
3 Grades of NHL Indolent (low grade, slow growing); Intermediate (aggressive, mix of small to large cells); High Grade (very aggressive)
NHL S/S Lymphadenopathy: persistent, painless, isolated or diffuse (retroperitoneum, mesentery, pelvis, extranodal – skin, GI tract); B symptoms: fever, night sweats, weight loss, (intermediate and high-grade dz); Abd pain, vomiting, bleeding, edema
NHL findings Normal CBC but poss anemia, thrombocytopenia, & leukopenia; occ lymphoma cells on diff; bulky lymphadenopathy (poss cause jaundice, hydronephrosis, SVC syndrome, bowel obstruction, wasting)
NHL staging CT chest, abdomen, pelvis; PET scan??; Unilateral or bilateral BM bx; LDH (tumor marker); LP if CNS dz is suspected
NHL mgmt Range of options from watch-and-wait to intensive chemo, with/without radiation; Tumor lysis prophylaxis (allopurinol, 300 mg/day, hydration, diuretics); BMT
NHL tx depends on: specific type and grade of NHL
Role of surgery in NHL Diagnostic
Mgmt of Low Grade NHL Watch & wait (“dynamic observation”) average of 6 yrs after dx; Rituximab (monoclonal antibody targets B-cells SAg CD20 cells), 1x/wk for 4 weeks; R-CHOP (rituximab plus cytoxan, adriamycin, vincristine, prednisone); CHOP
Mgmt of Intermediate Grade NHL Rituxan-CHOP; R-ICE; Bexxar (radiolabelled I-131); Zevalin[ radioimmunotherapy for NHL (radio-labelled rituxan)]
Mgmt of High Grade Lymphoma ((Large B-Cell Lymphoma) R-CHOP; R-ICE; many lymphomas tx’d w/specific tx’s (Burkett lymphoma, post transplant lymphoproliferative disorder); Lymphoblastic lymphomas are treated with regimens similar for T-cell ALL
Lymphoblastic lymphomas are treated with: Regimens similar for T-cell ALL
Oncologic emergencies Febrile Neutropenia; SVC Syndrome (superior vena cava syndrome); Tumor Lysis Syndrome; Hypercalcemia; Cord compression (myelomas)
Can affect T or B lymphocytes ALL
Type of HL that accounts for 80% of HL cases Nodular sclerosing HL
Bulky lymphadenopathy seen in: CLL; NHL
Only curative tx for MDS Allogeneic BMT
Smudge cells CLL
Serum viscosity 1.4-1.8 xH2O visc Waldenstrom’s
Next-door dz HL
Pancytopenia ALL, AML, hairy cell
Giant plts & teardrop poik Myelofibrosis
Drenching night sweats CLL, Hodgkins
B-symptoms fever, wt loss, drenching night sweats (HL, NHL)
Lymphadenopathy (in 80%) CLL
Skin infxn/shingles CLL
RAI staging is for CLL
Lytic lesions multi myeloma
Smudge cells CLL
Serum viscosity 1.4-1.8 xH2O visc Waldenstrom’s
Next-door dz HL
Pancytopenia ALL, AML, hairy cell
Giant plts & teardrop poik Myelofibrosis
Drenching night sweats CLL, Hodgkins
B-symptoms fever, wt loss, drenching night sweats (HL, NHL)
Lymphadenopathy (in 80%) CLL
Skin infxn/shingles CLL
RAI staging is for CLL
Lytic lesions MM
Auer rods AML
Dohle bodies blue patch near edge; PMN; infxn
Toxic gran purple/blue-black; PMN; severe infxn/toxic
Hyperseg 6or more/3% w/5 or more; PMN; megaloblastic anemia, B12-folate def
Vacuoles Monos/PMN; poss sepsis
BM aspirate iliac crest
LAP leukocyte alkaline phosphatase; Low in CML; High in leukemoid reactions
Meningitis AML/ALL
Fried egg hairy cell
Hypercellular bone marrow with reticulin or collagen fibrosis myelofibrosis
Preleukemia MDS
Immunoproferative dz Waldenström’s; Multiple Myeloma; MGUS
Plts in leukemias Increased (acute); normal (chronic)
Immunologic phenotypes = Common; Early B lineage; T cell ALL
M0-M7 phenotypes AML
Coexpression of CD19, CD5 CLL
Hypogammaglobulinemia CLL
Anemia seen in leukemias MM, hairy cell (NOTseen in CML)
Isolated lymphocytosis CLL
Lymphocyte pleomorphism; heterophile Ab EBV
Clustered bacteria in vacuoles in WBCs; low plts; leukopenia w/left shift; Rising Ab immunofluorescence titer Ehrlichiosis
Histoplasmosis CBC Organism in neutrophils & monocytes on Wright-Giemsa PBS; in macrophages on bone marrow smear
Anagrelide may be used for: P vera; thrombocytosis
Cytoreductive tx (eg, thrombocytosis); Anagrelide, hydroxyurea
Post showering pruritis, plethora, dyspnea, HA, visual disturbance, tinnitus; splenomegaly, engorged retinal veins; thromboses, high H&H Polycythemia vera; Tx: Phlebotomy
JAK-2 mutation P. vera (97% of pts); Essential Thrombocytosis (50%)
Engorged retinal veins seen in: P vera; waldenstrom
Created by: Adam Barnard Adam Barnard