luekemia + lymphoma + multiple myeloma Flashcards
tumor cells in bone marrow +/- peripheral blood
leukemia
tumor cells in lymph node
lymphoma
types of lymphoma
Hodgkin (1 type)
Non-Hodgkin (39 types)
Reed-sternberg cells: large cell, bi-lobed nucleus, prominent nucleoli with clearing “owl eyes”, clearing around whole cell (white)
MORE = WORSE prognosis
hodgkin lymphoma
will be on test!
localized, single group of nodes PAINLESS LAD (neck usually) mediastinal LAD (CT or CXR) firm, rubbery lymph nodes B symptoms: low grade fever, night sweats, weight loss (also in TB) pruritis associated with EBV: 50%
hodgkin lymphoma
bimodal age distribution 1st peak: 20 yo 2nd peak: 65 yo more common in men except nodular sclerosing type
hodgkin lymphoma
most are B cell lymphomas
Non-Hodgkin lymphoma
widespread lymph nodes + other tissues: GI, thyroid, CNS
fewer B symptoms
varying age
some associated with HIV and immunosupression
Non-Hodgkin lymphoma
types of hodgkin lymphomas
lymphocyte-predominant type
nodular sclerosing type
mixed cellularity type
lymphocyte-depleted type
hodgkin lymphoma
least common, BEST prognosis (mostly lymphocytes, not many Reed sternberg cells)
younger males
lymphocyte-predominant type
hodgkin lymphoma
most common
great prognosis (not many Reed sternberg cells)
F=M
young adults
bx: bands of collagen, sclerosis, fibrosis + nodules
nodular sclerosing type
hodgkin lymphoma
2nd most common
mix of lymphocytes + RS cells worse prognosis
mixed cellularity type
hodgkin lymphoma
lots of RS cells
very poor prognosis
lymphocyte-depleted type
types of non-hodgkin lymphoma (B cell)
diffuse large B cell lymphoma lymphoblastic follicular lymphoma burkitt lymphoma mantle cell lymphoma small lymphocytic lymphoma marginal cell lymphoma
most common type of NHL in adults
most common type of lymphoma in US
elderly
t (14;18): 30%
diffuse large B cell lymphoma
most common type of NHL in children
lymphoblastic lymphoma
NHL
t(14;18): 90%
follicular lymphoma
NHL
t(8;14): activates c-myc oncogene
“starry sky”: lymphocyte sheets (stars), some macrophages (sky) that have ingested apoptotic tumor cells
BurkiTT lymphoma (spells 8…14)
types of burkitt lymphoma
endemic: associated with EBV - Africa - jaw lesion
sporadic: pelvis or abdomen
immunodeficiency-associated: HIV
NHL
NHL
t (11;14): disrupts regulation of cyclin D →go into S phase quickly
mantle cell lymphoma
NHL
lymphoma equivalent of CLL
difference: tumor cells in lymph nodes
small lymphocytic lymphoma
NHL
arises in chronically inflamed tissue (outside lymph nodes)
associated with sjogren syndrome, hashimoto thyroiditis, H. pylori
marginal cell lymphoma
H. pylori = MALToma
types of non-hodgkin lymphoma (T-cell)
adult T cell lymphoma
mycosis fungoides/sezary syndrome
intestinal (enteropathy-associated) T cell lymphoma
NHL
caused by HTLV-1 (associated with IVDU)
cutaneous lesions
adult T cell lymphoma
NHL
cutaneous lesions
if malignant T cells leave skin lesions → blood: sezary syndrome
mycosis fungoides/sezary syndrome
NHL
associated with long term celiac disease (lymphocytes chronically in crypts)
intestinal (enteropathy-associated) T cell lymphoma
leukemia classifications
acute vs chronic
lymphoid (lymphocytes) vs myeloid (granulocytes: NEB) tumor cells
creates mutation bcr-abl (oncogene: mutated gene that encodes cancer, constitutively (always on) activated tyrosine kinase)
t(9;22): philadelphia chromosome
drug inhibits bcr-able tyrosine kinase
imatinib
activation of c-myc caused by
t(8;14)
t(9;22) seen in
CML-always
ALL- sometimes
AML - rare
t(8;14) seen in
BurkiTT lymphoma
t(15;17) seen in
M3 type of AML: has lots of Auer rods
drug to treat t(15;17) in M3 type of AML
all-trans retinoic acid
t(18;21) seen in
AML (trisomy 21: extra copy of 21, greater risk of translocation)
t(11;14) seen in
mantle cell lymphoma
t(14;18) seen in
follicular lymphoma
bcl-2 activation caused by
t(14;18)
cyclin D1 activation caused by
t(11;14)
↑ WBC count (>30-40,000) as result of stressor (infection!, down syndrome, tetrology of fallot, maligancy, kawasaki disease, heat stroke) mostly neutrophils left shift (5-10% immature bands)
leukemoid reaction
dysplasia of hematopoietic cells in myeloid tissue (like HPV)
pre-luekemic state: risk of transformation to AML
asymptomatic
↓CBC → bone marrow bx: dysplasia, disorderd hematopoiesis
“Pseudo-pelger-huet anomaly”: 2-lobed nuclei connected by thin strand
tx: monitor, give GF or transfusions as needed
myelodysplastic syndrome
myeloproliferative disorders: neoplastic transformation of single myeloid precursors → monoclonal proliferation of MATURE myeloid cells
polycythemia vera
essential thrombocytosis
myelofibrosis
CML: neoplasm of mature myeloid cells
from myeloid precursors
monocytes macrophages granulocytes: NEB erythrocytes megakaryocytes/PLT dendritic cells
from lymphoid precursors
T cell
B cell
NK cell
myeloid precursor cell acquires JAK2 mutation: constituitvely active janus kinase 2 → hematopoietic GF signaling to make more cells
polycythemia vera
essential thrombocytosis
myelofibrosis
JAK2 mutation in red cell precursor ↑ RBC mass + normal/low EPO plethora: reddened face pruritis after hot bath/shower splenomegaly hyperviscous blood: headache erythromelalgia: hands/feet are painful, swollen due to episodic clots in vessels
polycythemia vera
↑ PLT + low thrombopoietin
thrombosis or bleeding (defective PLTs)
essential thrombocytosis
fibrosis + obliteration of marrow space
“tear drop” RBC
myelofibrosis
vs adipocytes = aplastic anemia
most common tumor that arises in bone if > 50 yo
multiple myeloma
malignancy of plasma cells that arises in BM
↑ IgG (usually) or IgA
lymph node bx: plasma cells (large eccentric nuclei, chromatin gives “clock face” appearance, cytoplasm outside nucleus cleared out “perinuclear hof”)
monoclonal Ab spike on serum protein electrophoresis (SPEP): Monoclonal M spike
Bence Jones proteins: Ig light chains in urine detected on urine protein electrophoresis (UPEP) - BUT no ↑ protein on UA
punched out lytic bone lesions on XRAY (swiss cheese)
smear: RBC stacked like coins (rouleaux formation)
multiple myeloma
4 clues, >40-50 yo:
anemia: plasma cells interfere with production of other cell lines in BM
renal insufficiency: Ab casts in urine
back pain + hypercalcemia: secrete cytokines “local osteolytic factors” that stimulate osteoclasts, inhibit osteoblasts
multiple myeloma
associations with multiple myeloma
↑ susceptibility: lymphocytes are defective/deficient
1° amyloidosis (AL): deposition of Ig light chains
M spike (IgM) associated with amyloidosis + hyperviscous symptoms NO lytic bone lesions
waldenstrom macroglobulinemia
solid tumor of plasma cells
2 types: solitary plasmacytoma of bone or extramedullary plasmacytoma (usually head/neck, esp nose)
NO lytic bone lesions
plasmacytoma
monoclonal proliferation of plasma cells → production of monoclonal Ig (M spike)
No symptoms: no end organ damage yet
can progress to multiple myeloma (monitor)
monoclonal gammopathy of undetermined significance (MGUS)
