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Hematology & Oncology - Leukemia and lymphoma

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bomowoxu's version from 2016-11-02 15:04

Lymphomas and Multiple Myeloma

Question Answer
Leukemoid reactionAcute inflammatory response to infxn: ↑ WBC count with ↑ neutrophils and bands (left shift); ↑ leukocyte alkaline phosphatase
Contrast with CML (also ↑ WBC count with left shift,but ↓ leukocyte alkaline phosphatase)
Distinguishing features of Hodgkin's lymphomaLocalized, single group of nodes w/o extranodal involvement
Spreads continuously (stage is strongest prognostic indicator)
Reed-Sternberg cells
Young adults and > 55yrs - more common in men except for nodular sclerosing type
50% of cases assoc. w EBC
Constitutional signs/symptoms
Distinguishing features of non-Hodgkin's lymphomasMultiple, peripheral nodes w extranodal involvement
non-continguous spread (stage is irrelevant)
Majority involve B cells
20-40 y/o
Assoc. with HIV and immunosuppression
Fewer constitutional symptoms/signs
Reed-Sternberg cellsHodgkin's disease
"Owl's eyes"
CD30+ and CD15+ B-cell origin
Better prognosis w stromal or lymphocytic reaction against RS cells
Nodular sclerosing form: MC, best prognosis, men=women
Poor prognosis: lymphocyte mixed or depleted forms
Burkitt's lymphomaB cell NHL
Adolescents or young adults
t(8;14) translocation of c-myc (8) and heavy-chain Ig (14)
Assoc w EBV
Jaw lesions in African form - pelvic or abdomenal lesions in sporadic forms
Diffuse large B-cell lymphomaOlder adults, 20% kids
Most common adult NHL
Mainly B cell lymphoma, but 20% are T cell in origin
Mantle cell lymphomaB cell NHL
Older males
t(11;14) translocation of cyclin D1 (11) and heavy-chain Ig (14)
CD5+
poor prognosis
Follicular lymphomaB cell NHL
Adults
t(14;18) translocation of heavy-chain Ig (14) and bcl-2 (18)
Difficult to cure
Indolent course
bcl-2 inhibits apoptosis
Adult T-cell lymphomaT cell NHL
Adults
Caused by HTLV-1
Cutaneous lesions - esp. populations of Japan, West Africa, and Caribbean
Aggressive
Mycosis fungoides/Sezary syndromeT cell NHL
Adults
Cutaneous patches/nodules
CD4+
indolent course
Multiple myeloma is a neoplasm of what?Bone marrow that produces large amounts of IgG (55%) or IgA (25%)
"Fried egg" appearance
>40-50 y/o
Multiple myeloma is associated with?↑ susceptibility to infection
Primary amyloidosis (AL)
Punched-out lytic bone lesions on x-ray
M spike on protein electrophoreses
Ig light chains in urine (Bence Jones protein)
Rouleaux formation (RBCs stacked like poker chips in blood smear)
Smear of multiple myelomaNumerous plasma cells (clock-face appearance) with white intracytoplasmic inclusions containing immunoglobulin
Other than MM, what also has an M spike on protein electrophoresis?Waldenstrom's macroglobulinemia - but it's go hyperviscosity symptoms and no lytic bone lesions
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Leukemias

Question Answer
General pathophys of leukemiaunregulated growth of leukocytes in the BM → anemia (↓ RBCs), infxns (↓ mature WBCs), hemorrhage (↓ platelets)
Acute lymphblastic leukemia/lymphoma (ALL)Age: <15y/o
Mediastinal mass - Tcell ALL
Peripheral blood/BM have ↑↑↑ lymphoblasts
TdT+ (marker of pre-T and pre-B cells
CALLA+
most responsive to therapy
May spread to CNS/testes
t(12;21) → better prognosis
Small lymphocytic lymphoma (SLL)/chronic lymphocytic leukemia (CLL)Age: >60yrs
Asymptomatic - smudge cells in peripheral blood smear
Autoimmune hemolytic anemia
SLL=CLL except that CLL has ↑ peripheral blood lymphocytosis and BM involvement
Hairy cell leukemiaAdults - Mature B-cell tumor in the elderly
Cells have filamentous, hair-like projections
Stains TRAP (tartrate-resistant acid phosphatase) positive
Tx: cladribine, an adenosine anolog
Acute myelogenous leukemia (AML)~65 years old
Auer rods
↑↑↑ circulating myeloblasts on peripheral smear
t(15;17) → M3 AML subtype responds to all-trans retinoic acid (vitamin A) (induces differentiation of myeloblasts
DIC is a common presentation in M3 AML
Chronic myelogenous leukemia30-60y/o
Philadelphia chromosome (t[9;22]. bcr-abl)
↑ neutrophils, metamyelocytes, basophils
Splenomegaly
May acceperate and transform into AML or ALL ('blast crisis)
↓↓ leukocyte alkaline phosphatase d/t immature granulocytes (vs. leukemoid rxn - mature cells)
Tx: imatinib (inhibits bcr-able tyrosine kinase)
Auer rodsperoxidase-positive cytoplasmic inclusions in granulocytes and myeloblasts
AML (M3)
Tx can release Auer rods → DIC
Langerhans cell histiocytosisChild with lytic bone lesions and skin rash
Cells are immature and don't effectively stimulate Tcells via antigen presentation
Express S-100 (neural crest origin) and CD1a
Birbeck granules ("tennis rackets" on EM) are characteristic
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Leukemia/Lymphoma picture quiz

Question Answer
Reed-Sternberg cells → Hodgkin's lymphoma
Burkitt's lymphoma (non-hodkin's B cell lymphoma)
Multiple myeloma
ALL
SLL/CLL
Hairy cell leukemia
AML
CML
Langerhans cell histiocytosis
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Chronic myeloproliferative disorders

Question Answer
Polycythemia vera labs↑ RBCs, ↑ WBCs, ↑ platelets, Phili-chomosome, Positive JAK2 mutation
Essential thrombocytosis↑ platelets, negative Phili-chromosome, Positive (30-50%) JAK2 mutations
Myelofibrosis↓ RBCs, Variable WBCs, Variable platelets, negative Phili-chromosome, Positive (30-50%) JAK2 mutations
CML↓ RBCs, ↑ WBCs, ↑ platelets, Positive Phili-chromosome, Negative JAK2 mutation
Polycythemia vera pathABnormal clone of hematopoietic stem cells with contitutively active JAK2 receptors
Proliferate without EPO stimulation
Presents as intense itching after hot shower
Essential thrombocytosisMegakaryocytes with consitutively active JAK2 receptors that proliferate without EPO stimulation
MyelofibrosisFibrotic obliteration of bone marrow
Teardrop cell
CMLbcr-abl transformation leads to ↑ cell division and inhibition of apoptosis
Relative polycythemia↓ plasma volume
Appropriate absolute polycythemia↑ RBC mass, ↓ O2 saturation
Assoc w lung disease, congenital heart disease, high altitude
Inappropriate absolute polycythemia↑ RBC mass
Assoc w renal cell carcinoma, Wilms' tumor, cyst, heptocellular carcinoma, hydronephrosis
Due to ectopic erythropoietin
Polycythemia vera (Plasma volume and RBC mass)↑ plasma volume; ↑↑ RBC mass
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Rapid-fire: Leukemia/Lymphoma associations

Question Answer
KidsALL
T-cell leukemiaALL
↑↑↑ peripheral/bone marrow lymphoblastsALL
TdT+ALL
CALLA+ALL
CD10+ALL
Possible CNS/testes spreadALL
t(12;21) = good prognosisALL
>60 y/oCLL (maybe AML also)
smudge cells on smearCLL
autoimmune hemolytic anemiaCLL
B-cell leukemiaCLL & Hairy cell leukemia
hypogammaglobulinemiaCLL
TRAP+ (stain)Hairy cell leukemia
Tx w cladribineHairy cell leukemia
20-60 y/o (maybe older also)AML
auer rodsAML
↑↑↑ peripheral myeloblastsAML
t(15;17)AML
DICAML
tx w something that forces differentiationAML (all-trans retinoic acid)
40-60 y/oCML
↓↓ alkaline phosphatase levelsCML
t(9;22)CML
bcr-ablCML (it's a tyrosine kinase)
↑ PMNs, basophils, and metamyelocytesCML
tx w imatinibCML
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