LYMPHOMAS & LEUKEMIAS CHARACTERISTICS NON-HODGKINS LYMPHOMA (NHL): MATURE B-CELL LYMPHOMAS: - CLL/SLL accumulation of small lymphoid cells

in the peripheral blood, BM, and lymphoid organs - PB = SMUDGE CELLS - BM = Panocytopenia -2% of all leukemias - small B lymphs with abundant cytoplasm and hairy projections - found predominantly in BM and red pulp of spleen (beefy spleen) - BM has cells in an extracellular matrix of reticulin fibrils which cannot be aspirated causing a dry ta p - splenomegaly and pancytopenia in half the cases - Obliterates white pulp - originates from germinal centers and recapitulates follicular architecture - splenic white pulp involved - most common form of indolent NHL in U.S.* - most common form of NHL* - defining feature is the large cell size Dx: Based on site of involvement* ~ 60 (rare before 20) - CLL = PB, BM - SLL = primary LN, other lymphoid organs Middle-aged caucasian males 2-10 yrs survival - depends on extent of organ infiltration at time of Dx Very GOOD - very responsive to chemo regimens and produce long lasting remissions - CoD = infection - occurs at extranodal sites and spreads in an unpredictable pattern CLINICAL PRESENTATION/Dx Avg. ON SET (y/o) PROGNOSIS

- HAIRY CELL LEUKEMIA

- FOLLICULAR LYMPHOMAS

Painless, generalized lymphadenopathy

Middle-aged (males = females)

Incurable (median survival 7-9 yrs)

- DIFFUSE LARGE B-CELL LYMPHOMA

Enlarging mass at a nodal or extranodal site arising anywhere in the body e.g. Waldeyer ring - endemic jawbone mass - sporadic abdominal mass Dx: extremely important due to HIGH PROLIFERATION RATE

~ 60 (slight male predominence) - endemic young children - sporadic U.S. and Europe children and young adults HIV patients prognosis not favorable

- BURKITT LYMPHOMA - African (endemic) Lymphoma - Sporadic (nonendemic) Lymphoma - HIV+ individuals

- Diffuse proliferation with starry sky appearance from macrophages

MATURE T-CELL LYMPHOMAS: - MYCOSIS FUNGOIDES - most common cutaneous lymphoma* - small to medium lymphs with irregular nuclear outlines - these cells favor the epidermis but late disease spread to lymph nodes and BM - variant of MYCOSIS FUNGOIDES w/ associated LEUKEMIA - arises in a single node or chain of nodes and characteristically spreads to anatomical contiguous nodes - REED-STERNBERG CELLS* - Fever, night sweats, & weight loss - Painless lymphadenopathy - Spread - nodal disease, splenic disease, hepatic disease, finally BM involvement and other tissues - Staging is most important - I and II close to 90% cure rate - Advanced disease, disease free survival at 5 yrs is 60%70% Male = Female EXCELLENT

- SEZARY SYNDROME HODGKIN LYMPHOMA (HL):

- NODULAR SCLEROSING

- most common form* - presence of lacunar cell and collagen bands creating wellcircumscribed nodules - Reed Sternberg cells only occasional

- MIXED CELLULARITY - LYMPHOCYTE PREDOMINENCE (NON-CLASSICAL VARIANT HL) - LYMPHOCYTE RICH (CLASSIC HL)

- Lymph nodes have cellular infiltrate of small lymphs, eosinophils, plasma cells and macrophages mixed with RS cells - small lymphs admixed with benign histiocytes - have multilobed nucleus resembling popcorn kernel - RS difficult to find - reactive lymphs make up the cellular infiltrate - can be differentiated from lymphocyte predominance by presence of mononuclear variants and RS - EVB+ - least common

Males > Females Predominantly males (<35)

GOOD EXCELLENT

Very GOOD - EXCELLENT

- LYMPHOCYTE DEPLETED

Elderly & HIV+ predominent

LESS FAVORABLE

PB = Peripheral Blood RS cells = REED-STERNBERG Cells

LEUKOCYTE NEOPLASMS/DISORDERS CHARACTERISTICS/ETIOLOGY MYELOPROLIFERATIVE DISORDERS: - CML Increased production of 1+ types of blood cells - PHILADELPHIA CHROMOSOME (Translocation - 50-60 y/o of BCL-ABL gene)* - fatigue, fever, malaise, wt loss - Chronic = usually no blasts - splenomegaly - Increased marrow production of RBCs, granulocytes (basophils), platelets - Primary erythropoietin independent (EPO not high) - Secondary erythropoietin is high (can be caused by EPO secreting tumors - HCC) - WBCs greatly increased - myeloid cells from whole spectrum - may undergo blast crisis - distinguish from a leukemoid reaction - Headache, dizziness, HTN, gout, tired - total blood volume increased - Venous stasis - intense pruritis (basophils ) - Primary and Secondary - insidious early phase (2-3 yrs) INCIDENCE & S/S PERIPHERAL BLOOD/Dx PROGNOSIS/Tx

- POLYCYTHEMIA VERA - Primary - Secondary

MYELODYSPLASTIC SYNDROMES (MDS):

- Ineffective hematopoiesis leads to cytopenias - Younger patients --> BM Transplant - patients die from infection, bleeding - ETIOLOGY: proliferation of abnormal stem cells - Older patients --> supportive (cells are stuck in BM) therapy - Primary (idiopathic) - Secondary (radiation) - Can transform to AML

- 9-29 months (probably due to infection) '- Younger patients --> BM Transplant - Older patients --> supportive therapy

ACUTE LEUKEMIAS: - ALL - Most common CHILDHOOD CANCER* CHILDHOOD (2-10 yrs) - Pancytopenia - Lymphoblasts in Peripheral blood - 90% Remission (2-10 y/o) - ~60% Cured - Adult = POOR - Tx: Chemotherapy - difficult to treat - 60% remission - 15-20% remain free for 5 yrs

- AML

- Myeloid precursors accumulate in marrow - Proliferation of myeloid blasts - Auer Rods - Cytopenias

ADULTS (15-39 y/o)

- Pancytopenia - >30% Myeloid cells in BM = AML*