Lymphoma/leukemia Flashcards by Daniel Coquyt

Reed-Sternberg cells

Large cells
Bilobed nucleus
Prominent nucleoli w/ clearing around nucleoli – owl eye
Shrinks on slide -> clearing around cell

Assoc with Hodgkin lymphoma

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Hodgkin lymphoma

Reed-Sternberg cells

Single group of LN
Extranodal involvement rare

Painless, nontender LAD

neck
firm, rubbery

mediastinal LAD

Constitutional B Sx: low grade fever, night sweats, wt loss

Pruritis

Bimodal age: 20s and 65
Men > Women
Nodular sclerosing exception

Prognosis: more lymphocytes than RS cells good prognosis; more RS cells than lymphocytes, worse

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Lymphocyte-predominant type of HL

Least common
Best prognosis – few RS cells
Young males

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Nodular sclerosing type – HL

MC
Great prognosis – few RS cells

Bx: bands of collagen, sclerosis, fibrosis -> separates nodules

M=F
Young adults

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Mixed cellularity type of HL

2nd MC

worse prognosis

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Lymphocyte depleted type of HL

Lots of RS cells, not many lymphocytes

Very poor prognosis

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Non-hodgkin lymphoma

Most B cell lymphoma
LN and other tissues
Fewer constitutional sx
Variable age distribution
Certain types assoc w/ HIV and immunosuppression

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Adult T cell lymphoma

Aggressive, cutaneous

HTLV1

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Mycosis fungoides

Cutaneous
Lesions look like mushrooms
T cell lymphoma

T cells in blood – Sezary sn

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Diffuse large B cell lymphoma

20%
MC in adults, MC in US
Elderly

t(14;18) 30%

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Follicular lymphoma

T(14;18) – 90%

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Burkitt lymphoma

T(8;14) – activates c-Myc

Starry sky appearance – solid lymphocytes w/o M0 ingested tumor cells

Endemic – Africa
-assoc w/ EBV, involves mandible

Sporadic – pelvis, abdomen
Immunodef – assoc w/ HIV

B=8 and tt turns into 14

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Lymphoblastic lymphoma

MC in children

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Mantle cell lymphoma

T(11;14) – disrupts regulation of cyclin D -> S phase quickly

Connect tops of 1s to make an M and extend the 4 down for the A

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Small lymphocytic lymphoma

Lymphoma equivalent to CLL

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Marginal cell MALToma

Assoc w/ Sjogrens, Hashimoto thyroiditis, H pylori

Intestinal enteropathy assoc T cell lymphoma

Assoc w/ long term celiac dz

AML-M3 translocation

T(15;17)

Tx all trans retinoic acid

AML translocation

T(8;21)

Myelodysplastic syndromes

Dysplasia of myeloid hematopoietic cells
Risk -> AML

Peiger Huet anomaly
-N0 nuclei – two lobes connected with thin strand

asx – incidental finding

BM bx: dysplaysia
Disorderd hematopoiesis

Acute leukemia

Rapid onset and rapidly progressive

Over 20% myeloblasts (AML) or lymphoblasts (ALL) in bone marrow

Numerous blast (immature) cells more than 20% in blasts in blood

Often associated pancytopenia (anemia, bleeding tendency, infection)

ALL

Philadelphia chromosome may be seen – poor prognosis

MC in CHILDREN and young adults

M>F, W>AA

B cell types more common than T cell
3 morphologic variants and 5 phenotypic variants

Bone pain common

Very good prognosis in kids – up to 90% remission

Most have enzyme terminal deoxynucleotidyl transferase (TdT) +
PAS +

Difficult to dx on blood smear

Down Sn assoc

AML

ML-midlife
T(8;21)

Philadelphia chromosome RARELY seen

Auer rods – thin red/pink rods in cytoplasm of myeloblasts

M3 – acute promyelogenic leukemia t(15;17)

8 different morphological classifications

Most CD13/33+

Usually nonspecific esterase + myeloid cells

Median age of onset 65

PAS –
Assoc w/ numerous risk factors

AML risk factors

Radiation, benzene, or alkylating agents – HL tx

Myeloproliferative disease, myelodysplastic sn, or aplastic anemia

Down sn, Fanconi anemia, or Bloom Sn

Chronic leukemia

Insidious onset and gradual progression – months to years Mature cells rather than blasts (less than 5% blasts) Can be myeloid CML or lymphoid CLL Assoc w/ HSM and LAD Prominent infiltration of BM by leukemic cells Peripheral WBC count may be high

CLL

MC adult leukemia seen in western countries M>F, W>AA ELDERLY adults – median age 70 95% B cell markers rather than T cell 10% progress to ALL SMUDGE CELLS Autoimmune hemolytic anemia – warm and cold agglutinins Tends to be indolent Monitor for progression, same as small lymphocytic lymphoma

CML

May progress to AML 80% (blast crisis) or ALL 20% Numerous basophils and PMNs are LAP – Adults 25-60 yo, avg 55 Hyperplasia of all 3 cell lines (granulocytic, erythroid, megakaryocytic) but granulocyte precursors predominate T(9;22) ALWAYS present Fatigue, abd pain, splenomegaly, bleeding tendency

Philadelphia Chromosome

T(9;22) BCR-ABL -> constitutively activated tyrosine kinase “Philadelphia CreaM cheese” imatinib targets BCR-ABL tyrosine kinase

Multiple myeloma

MC arises w/in bone about 50 yo Monoclonal plasma cell cancer -IgG, sometimes IgA Anemia – plasma cells packed into BM interfere w/ other cell production Renal insufficiency – excessive Ab form casts in urine -Myeloma kidney Bone pain – esp back: local osteolytic factors – aggressive bone resorption Hypercalcemia M spike – monoclonal Ab spike on serum protein electrophoresis (SPEP) Bence Jones protein – Ig light chains in urine detected on urine protein electrophoresis (UPEP) -no increase protein on regular UA Increased susceptibility to infection Amyloidosis Xray: “punched out” lytic bone lesions Peripheral smear: Rouleaux formations – stacked RBCs

Walderstrom macroglobinemia

No lytic bone lesions IgM monoclonal proliferation Assoc w/ amyloidosis and hyperviscosity

Plasmacytoma

Solid tumor of plasma cells Types: - solitary plasmacytoma of bone - extramedullary plasmacytoma -> head and neck region – nose Do NOT cause lytic bone lesions

Monoclonal Gammopathy of undetermined significance (MGUS)

Monoclonal proliferation of plasma cells -> monoclonal Ig production No sx – no end organ damage yet! -> multiple myeloma eventually

Myeloproliferative neoplasms

Neoplastic transformation of a single myeloid precursor -> monoclonal proliferation of mature myeloid cells

JAK2 mutations

Polycythemia vera – RBC precursor -increased RBC mass w/o elevated EPO Essential thrombocytosis – thrombosis of bleeding -elevated platelets, low thrombopoietin Myelofibrosis – fibrosis and obliteration of marrow space -teardrop shaped red cells

Polycythemia vera

JAK2 mutation Plethora (red face) Pruritis after hot bath/shower Splenomegaly Hyperviscosity of blood – HA, erythromelalgia – painful hands