neoplastic proliferation of lymphoid cells that forms as a mass (in a node, or extranodal tissue)
Nodgkins & Non Hodgkins -within NON-hodgkins: B or T cell, size, patterns
(NON Hodgkins) Small B cell lymphomas (4)
follicular lymphoma mantle cell lymphoma marginal zone lymphoma small lymphocytic lymphoma
(NON Hodgkins) Intermediate sized B cell lymphoma (1)
(NON Hodgkins) Large B cell lymphoma (1)
Diffuse large B cell lymphoma
Burkitt's lymphoma -E?
-associated with EBV and with t(8;14) --> overexpression of c-myc oncogene
Burkitt's lymphoma -P?
-neoplastic proliferation of intermediate b cells (CD20+) that form an extranodal mass
Burkitt's lymphoma -L?
"starry sky" appearance with neoplastic B cells -reactive histiocytes and phagocytic debris = the stars
Burkitt's lymphoma -C?
-africa- jaw -america- GI tract, para-aortic nodes
Diffuse Large B cell lymphoma -E & P &C?
-neoplastic proliferation of large B cells that grow diffusely in sheets -the most common form of NHL -clinically aggressive -can arise sporadically or transform froma lower grade lymphoma -occurs in late adulthood as enlarging lymph node
Follicular Lymphoma -E?
neoplastic proliferationof small B cells (CD20+) that form follicle like nodules
Follicular Lymphoma -P?
d/t BCL2 on chromosome 19, translocates to the Ig heavy chain locus on chromosome 14 --> over expression of Bcl2 (which inhibits apoptosis)
Follicular lymphoma -Rx?
-reserved for symptomatic pts: rituximab, or low dose chemo
Marginal zone lymphoma -E & P?
-assoc w/ heliobacter pylori infection -neoplastic proliferation of small B cells that expand the marginal zone -can be MALToma = marginal zone lymphoma in the mucosa
Small lymphocytic lymphoma -E?
patients >60 -a neoplasm of small mature B cels SLL IF CONFINED to lymph nodes, = CLL if outside nodes
Hodgkins lymphoma -E?
bimodal peaks: 15-34 and >50 -often in the neck -assoc w/ EBV -B and T cells BECOME neoplastic REED STERNBERG cells
Reed sternberg cells=
large B cells with multi lobed nuclei and prominent nucleoli "owl eyed nuclei" that are mirror of one another and ar surrounded by a "halo" -usually CD15 and CD30 + DIAGNOSTIC of Hodgkins lymphoma
Types of Hodgkins lymphoma?
Nodular sclerosis (most common type!, also female dominant) Lymphocyte rich (best prognosis) Mixed cellularity (assoc w/ EBV, and high eosinophils) Lymphocyte-depleted (most aggressive, usually in old/hiv
Reedsternberg cells -secrete?
-can secrete cytokines which results in the "b" symptoms = fever, chills, and night sweats
Hodgkins lymphoma -C?
fever, unexplained weight loss, night sweats, pruritis, Pel Ebstein fever= alternatin gbouts of fever followed by remissions, painless enlargement of a single, or group of lymph nodes (often mediastinal or supraclavicular)
Langerhans Cell Histiocytoses -E?
-a proliferation of Langerhans cells -Langerhans are a subset of macrophages in the squamous epithelium, lymph nodes, spleen, and bronchiolar epithelium -they are antigen presenting cells but have undergone further differentiation. They have: CD1 + -contain Bierbeck granules (tennis racket appearance) -primarily occurs in children & young adults
Types of Langerhans Cell Histiocytoses?
Letterer-Siwe disease Hand -Schuller Christian disease Esosinophilic granulomas
-malignant histiocytosis -in kids -localized rash on scalp and in ear canals -diabetes insipidus s/t invasion of posterior pituitary -exopthalmos from infiltration of the orbit -lytic lesion in skull
-benign histiocytosis -often in adilescents -unifocal lytic lesions in bone (skull, ribs, femur) -bone pain and pathologic fratcures
Multiple Myeloma -E?
-balcks >whites -rare under 40 -Increased risk w/ benzene or radiation exposure -M spike in most cases
Multiple Myeloma -P?
-bone pain d/t "punched out lesions" in skull, vertebrae, ribs, pelvis; d/t interlukin 1 (activates osteoclasts) and inhibitor of osteoblast activation -hypercalcemia renal failure- has proteinaceous casts w/ multinucleated giant cell reaction
Why do encapsulated organisms thrive with dysfunction spleen?
-spleen is site of IgM synthesis, so the concentration of IgM drops = decreased complement activation -Macrophages are NOT present in spleen to phagocytose the opsonized encapsulated organisms -Loss of tuftsin, which normally synthesized in spleen, = decreased activation of macrophage receptors
Findings when spleen is malfunctioning?
-Howell Jolly bodies in RBC's = nuclear remnants becuase they weren't killed by spleen (even though defective) -infections with encapsulated organisms