33 terms

Lymphoid Tissue Disorders - Hematology

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Location of B cells?
-in germinal follicles in lymph nodes
-in the peripheral area of the splenic white pulp
Location of T cells?
- in the paracortex of lymph nodes
-in the periarteriolar sheath in the spleen
Left supraclavicular node enlargement d/t?
= Virchows nodes, metastatic stomach/pancreatic carcinoma
Hilar lymph node enlargement?
d/t metastatic lung cancer
lymphoma:
neoplastic proliferation of lymphoid cells that forms as a mass (in a node, or extranodal tissue)
Lymphoma classification?
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)
Burkitt lymphoma
(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
Letterer-Siwe disease
-malignant histiocytosis
-in infants/kids <2
-diffuse eczematous rash
-lytic lesions in sjull
-rapidly fatal :(
Hand-Schuller-Christian disease
-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
Eosinophilic granulomas
-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