Term
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Definition
Neoplasms of hematopoietic precursor cells in the lymphoid lineage, characterized by the accumulation of excess blasts in the bone marrow. |
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Term
prevalence of PreB vs PreT ALL |
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Definition
Precursor B-lymphoblastic ALL (Pre-B ALL) Majority of ALLs Occurs most frequently in childhood, but can also be seen in adults slightly more frequently in males than females
Precursor T-lymphoblastic ALL (Pre-T ALL) 15-25% of ALL occurs most frequently in late childhood, adolescence, and young adulthood male >female 2:1 |
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Term
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Definition
most = idiopathic Ionizing radiation Chemicals/drugs -benzene -chemotherapeutic agents (more frequently AML) Viruses -HTLV-1 (Human T cell Leukemia/Lymphoma Virus) : T-cell ALL -EBV: Burkitt’s Hereditary genetic associations -increased risk if sibling has acute leukemia Hematologic disorders predisposing to acute leukemia -CML (more frequently AML than ALL) |
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Term
6 broad categories of clinical signs of ALL |
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Definition
Rapid onset
Bone marrow infiltration -anemia -fatigue, pallor, -thrombocytopenia -bleeding, spontaneous bruising -neutropenia -infections, sepsis
Infiltration of other organs -lymphadenopathy -hepatosplenomegaly -mediastinal masses (T-ALL) -bone pain, esp. in children with ALL -skin -leukemia cutis -soft tissue -chloromas -testis -CNS leukostasis (only seen with WBC > 50 x 109/L) -CNS, strokes -lungs, pulmonary infiltrates, hypoxemia
Constitutional symptoms -fevers, sweats are common -weight loss uncommon
Coagulopathy -exposure of substances that can initiate coagulation, can cause DIC |
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Term
Lab features of ALL: BM biopsy, hematologic, chemistry |
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Definition
Hematologic WBC usually elevated, but can be normal or low normocytic anemia thrombocytopenia blasts are usually seen in the peripheral blood coagulopathy/DIC sometimes seen
Bone marrow biopsy hypercellular marrow excess number of lymphoblasts
Chemistry increased uric acid electrolytes abnormality increased LDH |
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Term
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Definition
Made by bone marrow biopsy
>20% lymphoblasts |
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Term
Histo features (see slide for picture!) |
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Definition
Cells with variable size and indistinct nucleoli
High nuclear/cytoplasmic ratio
Fine chromatin |
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Term
ALL vs lymphoblastic lymphoma DDx! |
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Definition
Clinically, a case is defined as lymphoma (LBL) if there is a mass lesion in the mediastinum or elsewhere and <25 percent blasts in the bone marrow.
It is classified as leukemia (ALL) if there are >25 percent bone marrow blasts, with or without a mass lesion.
It’s really the same disease with different presentations |
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Term
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Definition
light microscopy morphology AML blasts: Auer rods, cytoplasmic granules helpful if present. ALL blasts do not have Auer rods or granules. special stains (cytochemistry) Myeloperoxidase negative in ALL, positive in AML deoxytransferase (TdT) positive in ALL, negative in AML
flow cytometry Surface markers |
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Term
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Definition
Age
Cytogenetics Numerical changes Hyperdiploidy >50 chromosomes Translocations The Philadelphia chromosome: t(9;22), BCR/ABL: poor prognosis t(v;11q23); MLL rearranged: poor prognosis t(12;21)(p12;q22) TEL/AML1: a good prognosis and associated with hyperdiploidy |
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Term
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Definition
Chemotherapy Combination chemotherapy is the mainstay of treatment
Management of complications Infection – Abx Leukostasis – leukopheresis tumor lysis syndrome – hydration, allopurinol, rasburicase Allopurinol => reduced prd of uric acid…rasburicase breaks down uric acid too… Coagulopathy – supportive transfusion of red cells and platelets – cautious when leukostasis coexist
Supportive care Nutritional support Psychosocial support for patient and family |
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Term
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Definition
Remission induction – try to resume normal hematopo
Consolidation (intensification) – prevent relapse…
Maintenance – all px needs this! 1-2 yrs…
CNS prophylaxis – important for ALL more risk than AML…done by intrathecal therapy to get therapy into spinal fluid. |
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