LEUKEMIA www.freelivedoctor.com
“ leukoproliferative disorders” <ul><li>lymphoid lymphoid neoplasms acute </li></ul><ul><li>chronic </li></ul><ul><li>myel...
Lymphoid Neoplasms <ul><li>general aspects of lymphoid neoplasms </li></ul><ul><ul><li>1. histologic examination is requir...
Acute Leukemia <ul><li>monoclonal proliferation of immature “blast” cells that fail to participate in the normal maturatio...
Acute Leukemia <ul><li>general overview </li></ul><ul><ul><li>abrupt stormy onset </li></ul></ul><ul><ul><li>depression of...
Acute Leukemia <ul><li>- generalized lymphadenopathy </li></ul><ul><li>splenomegaly ALL > AML </li></ul><ul><li>hepatomega...
Acute Lymphocytic Leukemia/Lymphoma (ALL) <ul><li>40% of acute leukemia </li></ul><ul><li>children, young adults </li></ul...
Acute Lymphocytic Leukemia/Lymphoma (ALL) child ALL : almost curable adult ALL : recent improvement in prognosis, but stil...
Acute Lymphocytic Leukemia/Lymphoma (ALL) <ul><li>BM failure (pallor, lethargy, abnormal bleeding) </li></ul><ul><li>bone ...
Acute Lymphocytic Leukemia/Lymphoma (ALL) <ul><li>Peroxidase-granule negative (   AML) </li></ul><ul><li>terminal deoxynu...
ALL  peripheral blood smear <ul><li>normocytic, normochromic anemia </li></ul><ul><li>thrombocytopenia </li></ul><ul><li>W...
ALL  clinical course <ul><li>Remission in the majority of children </li></ul><ul><li>5 yrs disease free period in 50% of c...
ALL  prognostic factors <ul><li>good bad </li></ul><ul><li>age 2-10 y/o <1 y/o; adult </li></ul><ul><li>sex female male </...
“ leukoproliferative disorders” <ul><li>lymphoid lymphoid neoplasms acute </li></ul><ul><li>chronic </li></ul><ul><li>myel...
[Lymphoproliferative  Disorders] <ul><li>clonal expansions of antigen stimulated lymphocytes </li></ul><ul><li>maturation ...
Chronic Lymphocytic Leukemia (CLL) <ul><li>most common form of leukemia in North America and Northern Europe </li></ul><ul...
Chronic Lymphocytic Leukemia (CLL) <ul><li>Indolent clinical course </li></ul><ul><li>median survival : 4-6 yrs </li></ul>...
Hairy Cell Leukemia <ul><li>uncommon variant of  peripheral B-cell  neoplasm </li></ul><ul><li>clinically middle age to el...
Hairy Cell Leukemia  clinical <ul><li>M > F (3-5 : 1) </li></ul><ul><li>splenic red pulp involvement     red pulp “lake” ...
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Leukemia

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Leukemia

  1. 1. LEUKEMIA www.freelivedoctor.com
  2. 2. “ leukoproliferative disorders” <ul><li>lymphoid lymphoid neoplasms acute </li></ul><ul><li>chronic </li></ul><ul><li>myeloid myeloid neoplasms acute </li></ul><ul><li>chronic </li></ul>www.freelivedoctor.com
  3. 3. Lymphoid Neoplasms <ul><li>general aspects of lymphoid neoplasms </li></ul><ul><ul><li>1. histologic examination is required for diagnosis </li></ul></ul><ul><ul><li>2. wide range of behavior </li></ul></ul><ul><ul><li>3. majority are of B-cell origin (80-85%) </li></ul></ul><ul><ul><li>4. disruption of normal immune regulatory mechanisms </li></ul></ul><ul><ul><li>5. deriving from a single transformed cell (monoclonal) </li></ul></ul><ul><ul><li>6. homing to and growing in the areas of origin </li></ul></ul><ul><ul><li>7. tends to spread through lymphatics and peripheral blood to distant lymphoid tissue </li></ul></ul>www.freelivedoctor.com
  4. 4. Acute Leukemia <ul><li>monoclonal proliferation of immature “blast” cells that fail to participate in the normal maturation process </li></ul><ul><li>as the cells accumulate, they spill over into the peripheral blood </li></ul><ul><li>morbidity and death are rarely caused by leukemic cells </li></ul>www.freelivedoctor.com
  5. 5. Acute Leukemia <ul><li>general overview </li></ul><ul><ul><li>abrupt stormy onset </li></ul></ul><ul><ul><li>depression of marrow function </li></ul></ul><ul><ul><ul><ul><li>fatigue  anemia </li></ul></ul></ul></ul><ul><ul><ul><ul><li>fever, infection   WBC </li></ul></ul></ul></ul><ul><ul><ul><ul><li>bleeding   platelet </li></ul></ul></ul></ul><ul><ul><li>bone pain and tenderness  marrow expansion </li></ul></ul>www.freelivedoctor.com
  6. 6. Acute Leukemia <ul><li>- generalized lymphadenopathy </li></ul><ul><li>splenomegaly ALL > AML </li></ul><ul><li>hepatomegaly </li></ul><ul><li>- testicular involvement ALL > AML </li></ul><ul><li>- CNS involvement ALL > AML </li></ul><ul><li>headache </li></ul><ul><li>vomiting </li></ul><ul><li>nerve palsie </li></ul>www.freelivedoctor.com
  7. 7. Acute Lymphocytic Leukemia/Lymphoma (ALL) <ul><li>40% of acute leukemia </li></ul><ul><li>children, young adults </li></ul><ul><li>most frequent malignancy of childhood </li></ul><ul><li>80% of childhood acute leukemia </li></ul><ul><li>50% of cancer deaths in children <15 y/o </li></ul><ul><li>white : non-white = 2 : 1 </li></ul><ul><li>male > female </li></ul>www.freelivedoctor.com
  8. 8. Acute Lymphocytic Leukemia/Lymphoma (ALL) child ALL : almost curable adult ALL : recent improvement in prognosis, but still with significant mortality www.freelivedoctor.com pre B-cell pre T-cell Null cell 80-85% 15% <5%
  9. 9. Acute Lymphocytic Leukemia/Lymphoma (ALL) <ul><li>BM failure (pallor, lethargy, abnormal bleeding) </li></ul><ul><li>bone & joint pain </li></ul><ul><li>peripheral lymphadenopathy </li></ul><ul><li>splenomegaly </li></ul><ul><li>CNS involvement </li></ul><ul><ul><li>CSF : increased pressure and cellularity </li></ul></ul><ul><ul><li>decreased glucose </li></ul></ul><ul><ul><li>normal protein </li></ul></ul>www.freelivedoctor.com
  10. 10. Acute Lymphocytic Leukemia/Lymphoma (ALL) <ul><li>Peroxidase-granule negative (  AML) </li></ul><ul><li>terminal deoxynucleotidyl transferase (TdT) </li></ul><ul><li>common acute lymphoblastic leukemia antigen (CALLA) </li></ul><ul><li>T-cell antigen (T) --- mostly negative </li></ul><ul><li>surface Ig (sIg) --- mostly negative </li></ul><ul><li>cytoplasmic Ig (cIg) </li></ul><ul><li>PAS-pos cytoplasmic aggregate </li></ul>www.freelivedoctor.com
  11. 11. ALL peripheral blood smear <ul><li>normocytic, normochromic anemia </li></ul><ul><li>thrombocytopenia </li></ul><ul><li>WBC may be low, normal, or high </li></ul>peripheral B-cell pre B-cell pre T-cell www.freelivedoctor.com acid phosphatase PAS ++
  12. 12. ALL clinical course <ul><li>Remission in the majority of children </li></ul><ul><li>5 yrs disease free period in 50% of cases </li></ul><ul><li>complete cures possible </li></ul><ul><li>significant recent improvement in survival rate for T-cell type </li></ul>www.freelivedoctor.com
  13. 13. ALL prognostic factors <ul><li>good bad </li></ul><ul><li>age 2-10 y/o <1 y/o; adult </li></ul><ul><li>sex female male </li></ul><ul><li>WBC >50,000 blast/  L </li></ul><ul><li>phenotype early precursor B-cell mature B-cell, null cell </li></ul><ul><li>cytogenetics high hyperploidy pseudo-diploid </li></ul><ul><li>t(9;22) </li></ul><ul><li> 3% of pediatric ALL </li></ul><ul><li> 25% of adult ALL </li></ul><ul><li>t(8;14) </li></ul>www.freelivedoctor.com
  14. 14. “ leukoproliferative disorders” <ul><li>lymphoid lymphoid neoplasms acute </li></ul><ul><li>chronic </li></ul><ul><li>myeloid myeloid neoplasms acute </li></ul><ul><li>chronic </li></ul>www.freelivedoctor.com
  15. 15. [Lymphoproliferative Disorders] <ul><li>clonal expansions of antigen stimulated lymphocytes </li></ul><ul><li>maturation arrest due to molecular derangements </li></ul><ul><li>primarily B cell origin </li></ul><ul><li>may be induced by chromosomal translocations, retrovirus, or oncogene activation </li></ul><ul><ul><li>1. chronic lymphocytic leukemia (CLL) </li></ul></ul><ul><ul><li>2. hairy cell leukemia </li></ul></ul>www.freelivedoctor.com
  16. 16. Chronic Lymphocytic Leukemia (CLL) <ul><li>most common form of leukemia in North America and Northern Europe </li></ul><ul><li>essentially identical to small lymphocytic lymphoma (SLL) </li></ul><ul><li>M > F (2 : 1) </li></ul><ul><li>elderly (>60 y/o) </li></ul><ul><li>considered incurable </li></ul><ul><li>mostly asymptomatic </li></ul><ul><li>hepatosplenomegaly may be present </li></ul><ul><li>peripheral lymphocytosis (>200,000) </li></ul><ul><li>increased susceptibility to bacterial infection (most frequent cause of death) </li></ul><ul><li>may associated with autoimmune hemolytic anemia </li></ul>www.freelivedoctor.com
  17. 17. Chronic Lymphocytic Leukemia (CLL) <ul><li>Indolent clinical course </li></ul><ul><li>median survival : 4-6 yrs </li></ul><ul><li>occasional transformation to large non-Hodgkin’s lymphoma (Richter’s syndrome) --- 3 to 5 % </li></ul>www.freelivedoctor.com
  18. 18. Hairy Cell Leukemia <ul><li>uncommon variant of peripheral B-cell neoplasm </li></ul><ul><li>clinically middle age to elderly (younger than CLL) splenic red pulp involvement </li></ul><ul><li>histologically lymphocyte with finger-like projections </li></ul><ul><li>phenotypically TRAP (Tartrate Resistant Acid Phosphatase) </li></ul><ul><li>CD19, CD20 </li></ul>www.freelivedoctor.com
  19. 19. Hairy Cell Leukemia clinical <ul><li>M > F (3-5 : 1) </li></ul><ul><li>splenic red pulp involvement  red pulp “lake” </li></ul><ul><li>tends to follow an indolent course </li></ul><ul><ul><li>pancytopenia - most prominent feature </li></ul></ul><ul><ul><ul><li>- granulocytopenia  recurrent bacterial infection </li></ul></ul></ul><ul><ul><ul><li>- anemia  fatigue </li></ul></ul></ul><ul><ul><ul><li>- thrombocytopenia  bleeding </li></ul></ul></ul><ul><li>good response to some chemotherapy regimen </li></ul>www.freelivedoctor.com
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