Lymphoma/leukemia Flashcards
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
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
Lymphocyte-predominant type of HL
Least common
Best prognosis – few RS cells
Young males
Nodular sclerosing type – HL
MC
Great prognosis – few RS cells
Bx: bands of collagen, sclerosis, fibrosis -> separates nodules
M=F
Young adults
Mixed cellularity type of HL
2nd MC
worse prognosis
Lymphocyte depleted type of HL
Lots of RS cells, not many lymphocytes
Very poor prognosis
Non-hodgkin lymphoma
Most B cell lymphoma LN and other tissues Fewer constitutional sx Variable age distribution Certain types assoc w/ HIV and immunosuppression
Adult T cell lymphoma
Aggressive, cutaneous
HTLV1
Mycosis fungoides
Cutaneous
Lesions look like mushrooms
T cell lymphoma
T cells in blood – Sezary sn
Diffuse large B cell lymphoma
20%
MC in adults, MC in US
Elderly
t(14;18) 30%
Follicular lymphoma
T(14;18) – 90%
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
Lymphoblastic lymphoma
MC in children
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
Small lymphocytic lymphoma
Lymphoma equivalent to CLL
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
