L1a

Leukemia: 

 Leukemia: malignant proliferation of one or more elements of thehematopoietic system à primry BM involvement +/- circulating neoplastic cells in the peripheral blood

•       Acute vs chronic
•       Myeloid vs lymphoid

Leukemia involves neoplastic monoclonal proliferation of hematopoietic progenitor cells = blasts (acute leukemias) or their progeny (chronic leukemias)

-          Monoclonal cells are defined as a group of cells produced from a single ancestral cell by repeated cellular replica

Presentation and lab findings:

Clinical: -          Increase incidence with age (Except ALL = childhood & late adulthood – bimodal peak) -          M>F -          Symptomes: o   Bone pain o   Abdominal discomfort o   Headache o   LAD -          ↓normal blood cells o   Anemia à weakness, fatigue o   Neutropenia à infection/fever o   Thrombocytopenia à bleeding

Physical signs: -          Pallor -          Petechiae/purpura -          Bone tenderness -          Hepatosplenomegaly (HSM) -          LAD -          Gingival infiltrate (AML) -          Skin lesions

Lab findings: -          Aneima/neutropenia/ thrombocytopenia -          Circulating leukemia cells (usually) -          DIC + Coag abnormalitites -          Increased LDH/uric acid §  Typically seen in tumor lysis syndrome post Rx: break down produces of cells – can occur without Rx BM: replacement of normal cells with leukemic cells

Acute Vs. Chronic Leukemia

	Acute leukemia	Chronic leukemia
Onset	Abrupt	Slow/insidious
Progression	Rapid	Slow, indolent, relentless
Cell	Blasts = immature	Mature cells
Survival	Short (2-3 yrs) Except childhood ALL	Long

Answers to present case: AML

Bone marrow:

• The arrows are pointing to blasts
• In AML there will be >20% blasts

What is the arrow pointing to?

•        Auer rod: peroxidase positive cytoplasmic inclusions in granulocytes/myeloblasts – commonly seen in acute prolmylocytic leukemia 
•      Rx: can cause release of auer rods --> DIC

·         What translocation would support your diagnosis?

o   15;17 PML-RARa :surface receptor à affects transcription factors  à acute promyelocytic leukemia

·         Assuming Mr. Brown has this translocation what would you use to treat him?

o   All-transretinoic acid

·         Which of the following are positive cytogenetic markers for AML? ALL?

	Favorable	unfavorable
AML	8;21 15;17 16q22 breaks	6;9 Inv(3) (3;3) 11q23 breaks 5,7 abn
ALL	Hyperdiploidy 12;21	Hypodiploiidy 9;22 1;19 11q23

·         What are some of the risk factors for AML?

o   Down Syndrome

o   Other blood disorders (myelodysplasia, P. vera), smoking, exposure to radiation, benzene