Malignant Hematology

Introduction: Non-Hodgkin lymphoma is categorized into two groups of B-cell lymphomas and T-Cell lymphomas, B-cell lymphomas (85–90%): (discussed in a separate post B-Cell Non-Hodgkin lymphoma ) Low-grade B-cell lymphomas (LGBL): DLBCL: The most common, aggressive Follicular lymphoma (FL): Indolent, arising from germinal center B cells Marginal zone lymphoma (MZL): Extranodal, Nodal, Splenic Mantel cell lymphoma (MCL): More aggressive, Derived from mantle zone B cells Chroni

Monoclonal Gammopathies IgG >> IgM > IgA SLiM-CRAB: S: ≥ 60% BM Plasmacytosis Li: K/L ratio ≥ 100 or ≤0.01 M: MRI >1 focal lesion (>5 mm size) C: Calcium >11 or >1 ULN R: Renal Cr >2 or CrCl <40  A: Anemia Hb <10 or 2< LLN B: ≥ 1 lytic lesions MGUS:  M protein <3                                           Plasma cell in BM <10%          No SLiM-CRAB SMM:    M protein ≥ 3 or ≥ 500 mg/24hr urine     Plasma cell in BM 10-60%       No SLiM-CRAB MM:      Plasma cell proliferative d

Diffuse large B-cell lymphoma (DLBCL) Anti-CD19 ADC: Loncatuximab Tesirine Anti-CD19 CAR T cell: Preferred for relapsed/refractory DLBCL if relapsed <1 year. Tisag enlecleucel (tisa-cel) Axicabtagene ciloleucel (axi-cel) Lisocabtagene maraleucel (liso-cel) Brexucabtagene autoleucel (bre xu-cel) Anti-CD19 mAb: Tafasitamab Anti-CD79b ADC ( Antibody–Drug Conjugate ): Polatuzumab vedotin CD20 x CD3 Bispecific Ab: Engineered antibody that simultaneously binds to CD20 on B cells an

Background: Diagnosis is based on morphological evidence of dysplasia in BMBx Additional studies: karyotyping, flow cytometry, molecular genetics Pearl: Regardless of blast percentage, if a characteristic AML cytogenetic abnormality is seen on BMBx, then should treat as AML. Can be associated with previous drug exposures Example: alkylating chemotherapy or topoisomerase inhibitors Associated with del 5, del 7, or complex chromosomal abnormalities Risk Stratification: Based on

Here are some points that can make your Hematology/Oncology life easier- The earlier you learn them, the better.

Background: B-cell lymphoid malignancy, characterized by Reed-Sternberg cells ( resemble owl’s eyes ) Most commonly affecting young adults Presenting with painless LAP and sometimes B symptoms Types: Classic Hodgkin Lymphoma: CD15+, CD30+ and CD20- Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL): CD15-, CD30- and CD20+ Deaville Scoring System: Used to interpret FDG-PET scans for response assessment in HL and aggressive NHLs: 1 = No uptake 2 = Uptake ≤ mediastinum 3 =

Background: Allogeneic HSCT is generally reserved for high-risk or relapsed hematologic malignancies, while autologous HSCT is used for chemosensitive diseases such as multiple myeloma and certain lymphomas. Allo-HSCT: Infusion of hematopoietic cells from a HLA-compatible donor after cytotoxic conditioning to eradicate disease and enable engraftment. Common indications of HSCT in classic hematology: Severe aplastic anemia Hemoglobinopathies (sickle cell disease, β-thalassemia

Background: 10-15% of all leukemias Male/Female: 1.5 Median age of 65 years old Ionizing radiation is the only known causative factor (usually within 6 to 8 years of exposure) No known genetic factors determine susceptibility to CML Pathophysiology: >95% of CML patients have Philadelphia chromosome (Ph) in the marrow. Contains BCR-ABL1 fusion gene (translocation between BCR on chromosome 22 and ABL1 on chromosome 9)  BCR-ABL1 oncoprotein has tyrosine kinase activity. BCR-ABL1

Background: 25% of all leukemias Morphologically mature but immunologically incompetent B lymphocytes Incidence increase with age Male/Female: 2 Predominantly found in caucasians, less common in African-Americans, Hispanics and Asians Positive family history: 2-8 fold increased risk Work-up: CBC: Clonal B-lymphocytes >5000 Anemia and thrombocytopenia are common PBS:  Small lymphocytes with round nuclei, clumped chromatin, scant cytoplasm Medium/large sized cells: less than 10

Introduction: Non-Hodgkin lymphoma is categorized into two groups of B-cell lymphomas and T-Cell lymphomas: B-cell lymphomas (85–90%): High-grade B-cell lymphoma (HGBL): Diffuse large B-cell lymphoma (DLBCL) HGBL with MYC and BCL2/BCL6 rearrangements "Double-hit" lymphoma: MYC and BCL2 rearrangements "Triple-hit" lymphomas: MYC and BCL2 and BCL6 rearrangements Burkit lymphoma (BL): Highly aggressive, germinal center origin Low-grade B-cell lymphomas (LGBL): Follicular lymph

Background: Second most common acute leukemia in adults 75% of cases are B-cell lineage, 25% are T-cell lineage Work up: CBC BMBx: Cytogenetics, Molecular analyses Important to identify if presence of philadelphia chromosome or philadelphia-like chromosome Philadelphia-like chromosome: Lacks the BCR-ABL1 gene Associated with poor prognosis Consider use of TKIs and early transplantation Risk assessment: Favorable risk: Hyperdiploidy ETV6-RUNX1 Poor risk: Hypodiploidy (<44 chro