[8] HEMA - Chronic Leukemia (1).docx

**Hematology** **Chronic Leukemia** **[MPN]**   **Myeloid** **neoplasms** ---------- -- ------------- -- -- -- -- -- BCR-ABL1 gene is...

**Hematology** **Chronic Leukemia** **[MPN]**   **Myeloid** **neoplasms** ---------- -- ------------- -- -- -- -- -- BCR-ABL1 gene is Positive Accelerated ---------- -- ------------- -- -- -- -- --  Chronic Leukemia - Heterogeneous group of hematopoietic neoplasms. - Uncontrolled proliferation and decreased apoptotic activity with [variable degrees of] [differentiation] **(unregulated growth of myeloid cells in bone marrow and their** **accumulation in blood).** - Composed of relatively mature cells **(precursors are also present in circulation but** **mainly mature cells).** - Indolent (If untreated, the course is in months or years) **(patient could be** **asymptomatic for years).** - Occurs mainly in adults.   Lymphoid Myeloid Mixed Acute Lymphoid ---------- --------- -------------------- --------------------- ---------- -- -- undifferentiated ALL AML Acute biphenotypic Before formation of CLL (lymphpid + Myeloid and Myeloid) Lymphoid stem cells   - - -  - - - - - - Stem cell MPN (Different from Acute Myeloid Leukemia. How? As mentioned in page \#2, In AML, the pathogenesis is proliferation of Stem cells themselves (No differentiation) while in CML it leads to **increase in differentiation** of the stem cells - - Predominant proliferation of granulocytic cells. - Associated with the [BCR-ABL1] fusion gene located in the [Philadelphia (Ph)] chromosome which results from [t(9,22).]   - - - - - -- -- --------------------------------- -- -------------------- -- -- -- -- Adult Any age High High but \ - - - **High: Leukemoid**   -  **[Blood:] Pancytopenia with dysplasia**  **[BM]: Hypercellular with dysplasia** **Proliferation** ------------------- -- -- --  **Hematology** ----------------------------------------- -- -- **MDS has many subtypes according to:**   - Clonal hematopoietic malignancy characterized by proliferation of both monocytes & neutrophils. - MDS/MPN disease (Combination of both). - Philadelphia chromosome must be negative. - Blast must be less than 20% if 20% and above =\ acute leukemia. - Aggressive course: survival rate around 2.5 years. - Treatment chemotherapy ± SCT (stem cell transplantation).  **Hematology** **Chronic Leukemia** **Summary** - - - - - - - - - - - - - - - - -