Pathology - WBC Neoplasms Rapid Review

RAPID REVIEW DISEASE

KEY CONCEPTS

Large Granular Lymphocytic Leukemia

LYMPHOID NEOPLASMS PRECURSOR B AND T-CELL Most common cancer in children Hyperploidy (>50), hypoploidy, chromosomal aberrations Acute Lymphoblastic (-) MPO, (+) PAS Leukemia/Lymphoma 75-85% cure (ALL) Better prognosis: 2-10yo, low WBC, hyperploidy, trisomy 4,7, 10; t(12,21) Childhood; acute leukemia B-ALL TdT, CD19, CD10 Adolescent male, thymic lymphoma T-ALL TdT, CD2, 5, 7 PERIPHERAL B CELL CLL is most common leukemia in adults Deletions: 13q14.3, 11q, 17p; Trisomy 12q CD19, 20, 23, 5, surface Ig Proliferation Centers/Pseudofollicles Basket and Smudge cells CLL/SLL Poor prognosis: 4-6 years • 11q, 17p deletion; (+) ZAP-70 • Richter syndrome, Prolymphocytic transformation (3gm/dL), Bence-Jones protein (>6gm/dL) Variants that progress to MM: Solitary myeloma or plasmacytoma, Smoldering myeloma; MGUS Poor prognosis (4-6 years) Lymphoplasmacytic Deletion 6q Lymphoma IgM secretion Waldenstrom macroglobulinemia: hyperviscosity syndrome CD20, surface Ig PERIPHERAL T-CELL and NK CELL Peripheral T-Cell Wastebasket diagnosis; no pathognomonic lesions Lymphoma, Unspecified Anaplastic Large Cell Common in children and young adults; Lymphoma (ALK) rearrangements of ALK gene on 2p23 Good prognosis, 75-80% cure; Imatinib Adult T-cell Lymphoma HTLV-1: Japan, Caribbean, West Africa CD4 cells Cloverleaf/flower nuclei; Very fatal Mycosis Fungoides Three phases: premycotic, plaque, tumor Cerebriform nuclei in skin Sezary Syndrome Exfoliative dermatitis, leukemia (cerebriform nucleoid Sezary cells in PBS)

Extranodular NK/T-cell Lymphoma

HODGKIN LYMPHOMA

Rare (CD3) T cell and more common NK (CD56) variants Neutropenia and anemia Associated with rheumatologic disorders Common in Asia (Philippines), smokers, 20-40yo Commonly presents as nasopharyngeal mass Friable, prone to bleeding Aggressive but responsive to radiotherapy Reed-Sternberg Cells • Diagnostic or Owl’s Eyes • Lacunar Variant (Nodular sclerosis) • Mononuclear (Mixed Cellularity, Lymphocyte Rich) • Lymphistiocytic or Popcorn Cells (LymphocytePredominance) Ann Arbor Staging for prognosis, treatment Painless lymphadenopathy

CLASSICAL: CD15 and 30 Nodular Sclerosis Most common in young adults Cervical, supraclavicular, mediastinal nodes Lacunar and Diagnostic RS cells (-)PAX5 Mixed Cellularity Older, males; EBV implicated; B symptoms Heterogenous cells with diagnostic and mononuclear RS cells Lymphocyte-Rich Uncommon; mononuclear and diagnostic RS cells Lymphocyte-Depleted Least common form NONCLASSICAL: Males, usually younger than 35yo with Lymphocytecervical/axillary lymphadenopathy Predominance Type (+) CD20 and BCL6, B cell markers Lymphohistiocytic, popcorn variant MYELOID NEOPLASMS

Acute Myeloid Leukemia

Chronic Myeloid Leukemia

Myelodysplastic Syndrome

>20% immature blasts in BM; peak at 60yo Subtypes (M0-M7) M2/M3: Faggot cells with Auer Rods M3: DIC which can be treated by administration of transretinoic acid Good prognosis: t(8;21) or inv(16) 3 Leukocytosis (>100,000/mm )