RAPID REVIEW Large Granular Rare (CD3) T cell and more common NK (CD56)

DISEASE KEY CONCEPTS Lymphocytic Leukemia variants

LYMPHOID NEOPLASMS Neutropenia and anemia
Associated with rheumatologic disorders
PRECURSOR B AND T-CELL Extranodular NK/T-cell Common in Asia (Philippines), smokers, 20-40yo
Most common cancer in children Lymphoma Commonly presents as nasopharyngeal mass
Hyperploidy (>50), hypoploidy, chromosomal Friable, prone to bleeding
Acute Lymphoblastic aberrations Aggressive but responsive to radiotherapy
Leukemia/Lymphoma (-) MPO, (+) PAS HODGKIN LYMPHOMA Reed-Sternberg Cells
(ALL) 75-85% cure Diagnostic or Owls Eyes
Better prognosis: 2-10yo, low WBC, hyperploidy, Lacunar Variant (Nodular sclerosis)
trisomy 4,7, 10; t(12,21) Mononuclear (Mixed Cellularity, Lymphocyte
Childhood; acute leukemia Rich)
B-ALL
TdT, CD19, CD10 Lymphistiocytic or Popcorn Cells (Lymphocyte-
Adolescent male, thymic lymphoma Predominance)
T-ALL
TdT, CD2, 5, 7 Ann Arbor Staging for prognosis, treatment
PERIPHERAL B CELL Painless lymphadenopathy
CLL is most common leukemia in adults CLASSICAL: CD15 and 30
Deletions: 13q14.3, 11q, 17p; Trisomy 12q Nodular Sclerosis Most common in young adults
CD19, 20, 23, 5, surface Ig Cervical, supraclavicular, mediastinal nodes
Proliferation Centers/Pseudofollicles Lacunar and Diagnostic RS cells
CLL/SLL Basket and Smudge cells (-)PAX5
Poor prognosis: 4-6 years Mixed Cellularity Older, males; EBV implicated; B symptoms
11q, 17p deletion; (+) ZAP-70 Heterogenous cells with diagnostic and
Richter syndrome, Prolymphocytic mononuclear RS cells
transformation (<1 year) Lymphocyte-Rich Uncommon; mononuclear and diagnostic RS cells
Hairy Cell Leukemia Middle-aged, white, male Lymphocyte-Depleted Least common form
Pan B-cell markers, CD11c, 25, 103, surface IgG NONCLASSICAL: Males, usually younger than 35yo with
Cytoplasmic blebs Lymphocyte- cervical/axillary lymphadenopathy
TRAP test (tartrate resistant acid phosphatase) Predominance Type (+) CD20 and BCL6, B cell markers
Excellent; gentle chemotherapy (interferon) Lymphohistiocytic, popcorn variant
Diffuse Large B-cell Most common type of NHL; bimodal distribution;
MYELOID NEOPLASMS
Lymphoma (DLBCL) slight male predominance
BCL6 translocation >20% immature blasts in BM; peak at 60yo
CD19, 20, BCL6 Subtypes (M0-M7)
Primary effusion lymphoma (KSHV/HHV8) Acute Myeloid M2/M3: Faggot cells with Auer Rods
Immunodeficiency associated large B-cell Leukemia M3: DIC which can be treated by administration of
lymphoma (EBV) transretinoic acid
40-50% cured; fatal with no treatment; anti-CD20 Good prognosis: t(8;21) or inv(16)
3
Follicular Lymphoma Middle age (40-50yo) Leukocytosis (>100,000/mm )
t(14;18); BCL2 <10% blasts
CD19, 20, 10, BCL2, BCL6 Chronic Myeloid Fusion of BCR-ABL gene and Philadelphia
No macrophages; no apoptosis Leukemia chromosomes t(9;22)(q34;q11)
Incurable but indolent (7-9 years) Accelerated blast crisis
Burkitts Lymphoma Translocation in c-MYC, EBV implicated BM transplant, targeted therapy
Endemic (African; mandible); Sporadic (ileocecal) Not malignant but precursor for myeloid leukemia
are usually seen in children or young adults (10-40% transform to AML)
Myelodysplastic
HIV+ associated Maturation defects, mutated tyrosine kinases
Syndrome
CD19, 20, BCL6; (-) BCL2 Pawn ball megakaryocytes, nuclear budding,
Mantle Cell Lymphoma Elderly male Pseudo Pelger-Huet cells
Cyclin D1, CD5
3-4 years, no cure HISTIOCYTOSES
Marginal Zone Chronic inflammatory states (Hashimotos
Birbeck granules (tennis racket)
Lymphoma or thyroiditis, Sjogrens syndrome, etc)
Three Patterns
MALToma
Letterer-Siwe Disease: <2yrs, multifocal,
Multiple Myeloma Elderly, African descent
Langerhans multisystemic, skin lesions (tx: chemo)
Multifocal involvement of skeletal tissue
Histiocytoses Eosinophilic granuloma: unifocal
IL-6, abnormal secretion of M component, diverse
Hand-Schuller-Christian syndrome; invasion
chromosomal abnormalities
of posterior stalk of pituitary causing diabetes
M-protein spike (>3gm/dL), Bence-Jones protein
insipidus, exopthalmos
(>6gm/dL)
Variants that progress to MM: Solitary myeloma or
plasmacytoma, Smoldering myeloma; MGUS
Poor prognosis (4-6 years) NEOPLASMS OF AGE GROUPS
Lymphoplasmacytic Deletion 6q Children B-ALL
Lymphoma IgM secretion Endemic/Sporadic Burkitts
Waldenstrom macroglobulinemia: hyperviscosity Anaplastic Large Cell Lymphoma (ALK)
syndrome Adolescents T-ALL
CD20, surface Ig Hodgkin Lymphoma
PERIPHERAL T-CELL and NK CELL Young Adults Endemic/Sporadic Burkitts
Peripheral T-Cell Wastebasket diagnosis; no pathognomonic lesions DLBCL (slight male predominance; 20yo)
Lymphoma, Anaplastic Large Cell Lymphoma (ALK)
Unspecified Extranodal NK/T-cell Lymphomas (20-40yo)
Anaplastic Large Cell Common in children and young adults; Hodgkin Lymphoma (Nodular Sclerosis)
Lymphoma (ALK) rearrangements of ALK gene on 2p23 Adults CLL, CML
Good prognosis, 75-80% cure; Imatinib Middle Age Follicular Lymphoma
Adult T-cell Lymphoma HTLV-1: Japan, Caribbean, West Africa Hairy Cell Leukemia
CD4 cells Elderly DLBCL (slight male predominance; bimodal)
Cloverleaf/flower nuclei; Very fatal Mantle cell (male)
Mycosis Fungoides Three phases: premycotic, plaque, tumor Multiple Myeloma (African descent)
Cerebriform nuclei in skin Lymphoplasmacytic Lymphoma
Sezary Syndrome Exfoliative dermatitis, leukemia (cerebriform Acute Myeloid Leukemia
nucleoid Sezary cells in PBS) Myelodysplastic Syndrome