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68 Cards in this Set
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precursor lymphoblastic leukemia/lymphoma- cell lineages?
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-precursor lymphoblastic leukemia: B cells
-precursor lymphoblastic lymphoma- T cell |
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mature (peripheral) B cell lymphomas
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present at an older age
more common in males often involves bone marrow and peripheral blood (this gives it a more leukemic characteristic) small B cell: CD20 |
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know the WHO classification chart given at the beginning of the class
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*
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CLL/SLL
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-fairly common
-rare under age 40 -indolent (slow growing)depending on stage, difficult to completely cure -hypogammaglobulinemia, leads to : -increased infections |
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microscopic appearance of CLL/SLL
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lymphocytes are small and have a uniform appearance
"growth center"- where cells will grow out and progress to mature b cells |
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marginal zone lymphoma
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-most arise in post germinal center (pale area) memory B cells
SUBTYPES: -extra nodal MZL/MALToma (means arising in lymphoid aggregations throughout the body) -splenic marginal zone lymphoma -nodal marginal zone lymphoma |
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extranodal MZL/MALToma
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-gastric maltoma:may arise from helicobacter pylori
-also seen in some autoimmune diseases such as Hashimoto's thyroiditis(thyroid almost completely replaced by lymphoid tissue) and Sjogren's syndrome -slight female preponderence -can also be seen in breast, eye, and skin |
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splenic marginal zone lymphoma
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-cells may have villous projections
-older females -bone marrow and blood involvement -indolent -deletion in chrom 7q21, divides more rapidly. |
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nodal marginal zone lymphoma
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-monocytoid B cell component
-often has neutrophil infiltration-acute inflammation -marginal zone pattern -more aggressive than MALToma |
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microscopic appearance of lymph node in marginal zone B cell lymphoma
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architectural distortion, marginal zones are expanded. follicles are close together and impinging on each other
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follicular lymphoma
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-t(14,18)
-bcl-2 gene (protects the cell from apoptosis) -addition of other genetic problems inactivating TP53 deleting P38/MAPK -activation of C-MYC (these all promote cell growth) -grade divided 1)small or large cell 2)follicular and diffuse -low grade : follicular small cell -higher grade : diffuse large cell |
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mantle cell lymphoma
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(mantle refers to the outer ring of small lymphocytes surrounding a germinal center)
-elderly males -cells resemble the normal mantle zone b cells that surround germinal centers -aggressive -often extranodal involvement-lymphoid aggregates throughout the body -small cells -cyclin D, overexpression form t(11,14) -overcomes suppressor effects of retinoblastoma and p27 protein -survival 3-4 yrs |
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large b cell lymphoma
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- one of the most common forms of lymphoma
-broad age range -rapidly growing, usually symptomatic -mass at a single site -either nodal or extranodal -aggressive but curable -vesicular nuclei (bubbly), prominient nucleoli -basophilic cyto |
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LBCL-centoblastic
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-cleaved (cell has a little indentation) and non-cleaved large cells
-diffuse counterpart of nodular follicular lymphoma -more aggressive |
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LBCL- immunoblastic
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-large vesicular nucleus, prominent nucleolus, thick nuclear membrane
-nuclear hoff -intracytoplasmic immunoglobulin -seen in immunodeficiency, immunosupression, immune mediated diseases, hashimotos, sjogren's syndrome, SLE |
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LBCL- Tcell/ histiocyte rich
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-may see Tcells and histocytes (tissue macrophage)
-diffuse growth pattern with fine interstitial fibrosis |
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LBCL- ananplastic
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-rare variant
-large bizzarre tumor cells |
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clinical presentation of LBCL
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-primary mediastinal (thymic) B cell lymphoma- respiratory problems, SVC syndrome, swelling of head and neck
-primary CNS lymphoma- ass. w HIV. may have seizures or changes in personality -primary effusion lymphoma- ass. w/ body cavities |
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primary mediastinal (thymic) LBCL
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-women in their 30's
-SVC syndrome -airway obstruction -locally invasive -multilobulated cells |
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Primary CNS lymphoma
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-ass. w HIV and Epstein Barr
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Primary effusion lymphoma
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-HIV ass. lymphoma
-caused by HHV-8 |
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burkitt lymphoma
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-homogeneous medium sized cells, rounded nuclei, multiple nucleoli, basophilic cytoplasm
-cytoplasmic lipid vacuoles -multiple large macrophages -"starry sky" pattern: macrophages are "stars" and the lymphocytes make up the "sky" -rapid cell turnover, proliferation and apoptosis -seen in HIV, also in children in Africa |
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burkitt lymphoma cont.
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-mature B-cell express CD10 and BCL6
-C-MYC rearrangement and overexpression resulting in cell cycle -t(8, 14) translocation |
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subtypes of burkitt lymphoma
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-endemic: jaw and facial bones, EBV 100%
-non-endemic: abdominopelvie region, terminal illeum, cecum, ovaries -immunodeficiency:systemic, extranodal, not in CNS, EBV 30% -all are aggressive |
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peripheral T-cell lymphomas
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-less common than B-cell
-can be more aggressive than B -some have a very poor prog -others have a favorable prog -high incidence of bm involvement -small lymphocytic type -mixed cell type -large cell/immunoblastic type |
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adult T-cell lymphoma/leukemia
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-rare in us, common in Japan
-skin lesions -hypercalcemia -lytic bone lesions:osteoclastic activity -aggressive -ass. w/ human retrovirus HTLV1 |
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anaplastic large cell lymphoma
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-more common in young adults
-involves lymph nodes and marrow -aggressive but responds to chemo -cell with large eccentric nuclei with prominent nucleoli -may express antigen ALK1 |
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angioblastic T-cell lymphoma
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-presents with advanced disease
-bone marrow involvement is 50-70% -systemic symptoms -prominent vascularity -mixture of plasma cells, lymphocytes, eosinophils -aggressive course |
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mycosis fungoides
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-adults
-multiple skin lesions: patch, plaque, tumor -papillary dermal fibrosis -Pautrier's microabscessses -cells with cerebriform nuclei -indolent but progressive course |
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sezary syndrome
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mycosis fungoides with generalized adenopathy and cerebriform cells in the circulation
-unfavorable prog |
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Hepatosplenic T-cell lymphoma
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-hepatosplenomegaly
-chrom 8 trisome -young black males -monotonous small to medium sized cells -sinusoidal infiltration of liver, spleen, and marrow -aggressive course |
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hodgkin disease characteristics
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-single node or chain of nodes
-reed-sternberg cells:neoplastic giant cells -systemic symptoms: fever, night sweats |
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4 types of hodgkins
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-lymphocyte predominance
-mixed cellularity -lymphocyte depletion -nodular sclerosis |
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reed-sternberg cells
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multilobate nucleus or multinucleate with large nucleoli, big googly eyes
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hodgkins may be ass. w/
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EBV
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Hodgkins disease- lymphocyte predominance
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-large number of lymphocytes mixed w/ histiocytes
-popcorn cells: R-S variants with multilobed puffy nuclei -excellent prog |
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hodgkins disease- mixed cellularity
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-more males, over 50
-plentiful R-S cells -heterogeneous cellular infiltrate -presents more often with disseminated disease |
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hodgkins disease- lymphocyte depletion
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-least common form of HD
-scarse lymphocytes, many R-S cells -most older pts, disseminated disease |
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hodgkins disease- nodular sclerosis
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-most common form
-lacunar cells : R-S variant, hyperlobate nuclei, multiple nucleoli, surrounded by clear spaces -collagen bands divide lymphoid tissue into nodules |
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more about nodular sclerosis hodgkins
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more women than men
-involves lower cervical, supraclavicular, and mediastinal LN -most pts are adolescents or young adults -excellent prog |
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hodgkins clinical course
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-histologic characteristic has little impact on prog, stage is what is important
-chemo-rad protocols increase risk of other maligs |
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hodgkins stages
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I- 1 lymph node
II-2 or more nodes on same side of dia III- lymph nodes on both side of dia IV- diffuse involvement of 1 or more extralymphatic organs with or without ass lymph node involvement |
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benign follicular hyperplasia
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-vary in size shape and number of cells
seen in children and adolescents |
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tingible body macrophages
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often seen in benign follicular hyperplasia, come in to clean up lymphocytes as they apostosis
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Toxoplasmosis lymphadenitis
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-Unilateral posterior lymph nodes
-Reactive follicular hyperplasia -Small aggregates of histiocytes - B-cell hyperplasia -Epithelioid clusters encroach on follicles -Monocytoid B-cells seen in subcapsular and trabecular sinuses -AIDS |
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Follicular hyperplasia-syphilis
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-Has periarteritis (surrounds blood vessels)
-Epithelial histiocytes -Thick capsule -Plasmacytic and lymphocytic infiltrate (attracts PLASMA cells) -Spirochetes present in the wall of the blood vessels -Solitary inquinal lymphadenopathy |
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Granulomatous lymphadenitis- types of
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-Kikuchi disease
-Cat scratch disease -Lymphogranuloma venereum -Sarcoidosis |
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Kikuchi disease
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-Young women
-Fever -similar to SLE -Necrosis and aggregates of macrophages with “C” shaped nuclei and CD8+ immunoblasts in interfollicular areas -Necrotic areas with debris and sparse neutrophils -Plasmacytoid monocytes with round nuclei and eosinophilic cytoplasm |
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Sarcoidosis
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-Discrete well circumscribed epitheliod granulomas
-Asteroid bodies -Schaumen bodies -Calcium oxalate crystals -Diagnosis of exclusion |
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Cat scratch disease
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-Suppurative granulomas with stellate abscesses surrounded by pallisading macrophages and histiocytes especially in germinal centers
-follicular hyperplasia -bartonella henselae: seen in early stages -Bacillary angiomatosis caused by same organism but in immunocompromised hosts with dermal lesions, increased capillaries,plump endothelial cells, granular eosinophilic material containing the organism |
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Lymphogranuloma venereum
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-Similar to cat scratch disease
-Negative silver stain -Presence of chlamydia |
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HIV related hyperplasia
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-Extravasation of RBC’s into the germinal centers
-Thinning and elimination of mantle zones -Warthin-Finkeldey: cells with multiple nuclei and eosinophilic intranuclear inclusions -Stain for HIV core protein P24 in germinal centers |
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HIV related hyperplasia cont.
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-As immunosuppression progresses pattern evolves from follicular hyperplasia to follicular involution to lymphocyte depletion
-Follicular hyperplasia florid and termed explosive -Large irregular follicles which can coalesce to form geographic follicles |
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cheat sheet
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stellate abscess- cat scratch
geographic follicles- HIV plasma cells, periarteritis - syphilis *know these |
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Diffuse pattern- causes
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-Infectious mononucleosis
-Postvaccinial lymphadenitis -Dilantin hypersensitivity |
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Infectious mononucleosis
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-May resemble lymphoma
-Reactive follicles with marked interfollicular expansion -Many immunoblasts and lymphocytes give a mottled appearance -Cell may resemble Reed Sternberg cells |
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Post vaccinial lymphadenitis
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-Used to be seen with smallpox
-Resembles infectious mononucleosis -Mottled vascular mixed cell infiltrate |
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Dilantin hypersensitivity
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-Fever, erythematous rash and eosinophilia
-Immunoblasts, eosinophils and plasma cells |
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Follicular hyperplasia-RA
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-Interfollicular plasmacytosis similar to syphilis
-May have Russel bodies -Increased risk of lymphoma |
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Castleman Disease:
hyaline vascular type |
-more common,
-Mantle zone hyperplasia with concentric layering of cells giving an onionskin pattern -Vessels penetrate into the germinal center resembling a “lolipop” -Two or more germinal centers -Hyalinized vessels |
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Castleman disease- plasma cell type
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-Plasma cell type: sheets of plasma cells
-Plasma cell type often asymptomatic (you can also have Multicentric Castleman: aggressive, linked to herpes virus 8) |
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Sinus pattern
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Non specific pattern
Rosai-Dorfman syndrome Whipple disease |
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Rosai-Dorfman syndrome
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-AKA sinus histiocytosis with massive lymphadenopathy
-B/L nontender cervical nodes -First two decades of life -Dilated sinusitis -Histiocytes, S100+,macrophages with emperipolesis -Fevers , neutrophilia, elevated ESR -Nodes matted together from fibrosis |
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Whipple Disease
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foamy macros
bacillary orgs (sickle shaped) malabsorption- anemic, tetany, easy bruisiblity, CNS changes, hyperpigmentation |
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Virus associated hemophagic syndrome
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phagocytosis of RBCS (also neuts and plates)
children can look like leukemia, pancytopenia |
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dermatopathic lymphadenitis
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-ass w exfoliative dermatitis
-expansion of large cells around follicles and cortex, pigment laden macrophages may look like mycosis fungoides |
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ALPS
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Autoimmune lymphoproliferative syndrome Lymphadenopathy, -splenomegaly
Hypergammaglobulinemia Autoimmune features Genetic type mutation of FAS(CD95),generally activates apoptosis inc risk of lymphoma |
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Kawasaki disease
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kids
Conjunctivitis,coronary artery involvement Nodal architecture obliterated Lymphoid depletion and absence of follicles Foci of necrosis and thrombi in vessels |