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Leukemias
Hematology
| Question | Answer |
|---|---|
| Most commonly occurring leukemia | CLL |
| CLL Mgmt | Observation; chemo (fludarabine & cladribine. cyclophosphamide, Rituximab); BMT; tumor lysis prophylaxis (allopurinol, 300 mg/day, hydration, diuretics); radiation (vs 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; HSM; LAD (80%); wt loss; frequent/persistent infxn; skin infxn/shingles |
| Anemia & thrombocytopenia; mature small lymphs & smudge cells; Hepatomegaly; Splenomegaly - seen in: | CLL findings |
| 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 | Acute phase, when blasts comprise >30% of BM cells. Pts with CML that evolves into acute phase have poor response to tx & die in this phase. |
| CML will likely transform to: | Acute disease |
| Primarily disease of children | ALL |
| What percentage of ALL patients are children (usually 3 -7 yo) | 80% |
| Accounts for 10-20% of acute adult leukemia | ALL |
| CML mgmt | Tyrosine kinase inhibitors (Gleevec/imatinib; allogeneic SCT; Dasatinib, AMN107; interferon & hydroxyurea |
| 3 phases of CML are defined by: | blasts in marrow |
| Slowly progressing disease: too many WBCs made in BM (especially myeloid cells) = | CML |
| CML prevalence | Young to middle age adults (median 45-55 yo); 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 Sx/Sx | Fever (w/o infxn); bone pain; LUQ pain (enlarged spleen); night sweats; bleeding & bruising; petechiae; fatigue ; weakness |
| 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 Sx/Sx | Meningitis; fever (abrupt onset with children); petechiae; anorexia, gingival hypertrophy. DIC, retinal hemorrhages. Leukemia cutis (skin) in AML. |
| ALL & AML labs | Pancytopenia; hyperleukocytosis; BM >20% blasts |
| AML: median age at onset: | 65 yr (AML = 80% of all adult-onset acute leukemias) |
| 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 | Possibly exposure to toxins (benzenes, radiation, chemotherapy) |
| AML findings | Pancytopenia, circulating blasts, often WBC >200,000; BM >20% blasts; Auer Rods; high ESR; hepatosplenomegaly |
| AML mgmt | Chemo; Induction & 3 consolidation treatments, in hospital. Induction (ARA-C with mitoxantrone, idarubicin, or daunorubicin). Post-induction: chemo vs SCT. 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 |
| Disease treated w/tumor lysis Ppx | AML &CLL & NHL |
| Hallmark of Hairy Cell leukemia | Pancytopenia; “hairy” cells |
| Bulky lymphadenopathy seen in: | CLL; NHL |
| Next-door disease | HL |
| B-symptoms | fever, wt loss, drenching night sweats (HL, NHL) |
| Drenching night sweats seen in: | CLL, Hodgkins |
| Can affect T or B lymphocytes | ALL |
| Pancytopenia seen in: | ALL, AML, hairy cell |
| Lymphadenopathy seen in in 80% of patients with: | CLL |
| Skin infection/shingles | CLL |
| RAI staging is for | CLL |
| Meningitis seen in: | AML/ALL |
| Immunologic phenotypes include: Common; Early B lineage; T cell | ALL |
| M0-M7 phenotypes (based on degree of differentiation & maturation of predominant cells) used to classify: | AML |
| Co-expression of CD19, CD5 | CLL |
| Hypogammaglobulinemia | CLL |
| Anemia is seen in which leukemias | MM, hairy cell (NOT seen in CML) |
| Isolated lymphocytosis | CLL |
| Malignant proliferation of lymphoid stem cells in BM -> invade LNs, spleen, liver = | ALL |
| Factors associated with worse prognosis in adults with ALL | Age >60, Philadelphia chromosome-positive, long time to first remission, high WBC at time of dx |
| Binet Stage A = | CLL: lymphocytosis with <3 LN groups, no anemia or thrombocytopenia |
| Binet Stage B = | CLL: lymphocytosis with >3 LN groups |
| Binet Stage C = | CLL: anemia / thrombocytopenia +/- LN groups |
| Caused by proliferation of abnormal myeloid cells that do not mature | AML |
| AML may be preceded by: | CML, P vera, idiopathic myelofibrosis, or MDS |
| Caused by proliferatin of myeloid cells that retain their capacity to differentiate | CML |
| Philadelphia chromosome-negative CML is associated with: | poor prognosis |
| bcr / abl gene | CML |
| Leukemias associated with Philadelphia chromosome | CML (OK prognosis; if Phila chromosome-neg: poor prog). ALL (poor prognosis) |
| Auer rods / peroxidase | AML (not seen in ALL) |
| Pancytopenia with blasts: | ALL |
Created by:
Abarnard
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