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44 Cards in this Set
- Front
- Back
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Caseating granulomas
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Tuberculosis; some fungi–Coccidioidomycosis
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Noncaseating granulomas
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Sarcoidosis; early TB
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Acute Lymphadenitis
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enlarged, tender LN
•Morphologic types: Follicular hyperplasia and PMNs : pyogenic infection • Follicular hyperplasia and suppurative granulomas - Cat-Scratch disease (Bartonella) - Tularemia - Lymphogranuloma venereum; Yersinia |
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Mesenteric lymphadenitis
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Acute lymphadenitis involving numerous intraabdominal lymph nodes draining the intestines
Symptoms mimic acute appendicitis |
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Chronic nonspecific lymphadenitis
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• Three morphologic patterns
‐ Follicular hyperplasia ‐ Paracortical hyperplasia ‐ Sinus histiocytosis |
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Follicular Hyperplasia
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B cell proliferation results in prominent
germinal centers; apoptotic bodies ‐ HIV (early) ‐ Rheumatoid arthritis ‐ Drug reactions; some viral infections |
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Paracortical‐Interfollicular Hyperplasia
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•T-cell hyperplasia of immunoblasts with
infiltrate of macrophages and eosinophils •Associations: viral infections, esp. infectious mononucleosis, CMV, varicella-zoster •Vaccinations •SLE (associated with necrosis |
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Sinus Histiocytosis
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Increase in macrophages
(histiocytes) in subcapsular and trabecular sinuses - Common in nodes draining cancers, eg breast, colon |
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Lymphoid Neoplasms, gen info
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• Lymphomas are neoplastic monoclonal
proliferations of lymphocytes that form a mass • All lymphomas are considered malignant • B‐cell origin for 80‐85% of lymphoid neoplasms ‐ T‐cells: most of remainder ‐ NK, histiocytic neoplasms rare • Lymphoma causes immunodeficiency • Lymphoma may produce autoimmune disease |
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Follicular Lymphoma
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NHL in adults in US
• Immunophenotype: B‐cell markers CD19, CD20, CD10 • t(14;18) ‐‐> overexpression of BCL2 ‐‐ blocks apoptosis |
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morphology of follicular lymphoma
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‐ Lymph nodes enlarged, nodular; matted masses
‐ Round to oval follicles or nodules |
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Follicular lymphoma: Clinical
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• Painless, generalized lymphadenopathy
• Poorly responsive to chemotherapy • Indolent: 7‐9 year median survival • Transformation to aggressive large B lymphoma occurs in half: survival < 1 year |
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Diffuse Large B‐cell Lymphoma, gen
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• Aggressive
‐ All ages ‐ Most common aggressive lymphoma of adults • Immunophenotype/genetics ‐ B‐cell markers: CD 19, CD 20 positive - bcl – 6 transcription factor most common |
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Special types of large B cell lymphoma
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• Immunodeficiency‐associated
- HIV, xplant, congenital, EBV • Body cavity large cell type lymphoma - advanced HIV, malignant cells c HH8 virus |
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treatment for large B cell lymphoma
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intensive chemotherapy; remission
in 80%; half are cured |
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morphology of Burkitt Lymphoma
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‐ Diffuse, intermediate uniform lymphs with a starry sky pattern of apoptotic bodies
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the 3 types of Burkitt Lymphoma
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‐ African (endemic) Burkitt
‐ Sporadic (nonendemic) Burkitt ‐ Burkitt Lymphoma associated with HIV |
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Burkitt lymphoma, immunophenotype
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‐ B‐cell markers: CD19, CD 20, CD 10
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Burkitt lymphoma cytogenics
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chromosome 8, myc gene
translocation involved all cases - t(8:14) IgH locus |
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clinical aspects of African Burkitt lymphoma
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‐100% association with Epstein Barr virus (EBV)
‐ Tends to involve mandible |
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Burkitt lymphoma in HIV
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‐ 25% associated with Epstein Barr Virus
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Sporadic Burkitt lymphoma in the US
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- not typically EBV
‐ Causes 30% of childhood NHL; 5% of ALL ‐ Ileocecal mass is a most common site in children ‐ Other visceral involvement: kidneys, ovaries, adrenal glands - responsive to chemotherapy |
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Mantle Cell lymphoma
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NHL
• Naïve B‐cell; resembles follicular lymphoma and CLL • t(11;14) ‐‐> bcl‐1 (cyclin D1 gene): controls cell cycle; CD19, CD20 + |
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clinical- mantle cell lymphoma
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‐ Middle age; M > Fe
‐ Generalized lymphadenopathy with spleen and bone marrow involvement ‐ Survival 3‐4 years |
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MALToma
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• Occurs in middle‐aged adults
• Nodal disease: marginal zone lymphoma • Extranodal types‐ characteristics: ‐ Tend to arise in tissues involved by chronic inflammation, infection ‐ Remain localized for prolonged periods; late systemic spread ‐ May regress if inciting agent is eradicated |
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what are Extranodal Marginal Zone Lymphomas associated with
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* autoimmune diseases
‐ Salivary glands‐Sjogren’s syndrome ‐ Thyroid‐Hashimoto’s thyroiditis • chronic infection ‐ Helicobactor pylori gastritis |
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pathogenisis of MALToma
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‐ Reactive polyclonal proliferation
‐ Monoclonal transformation; evolves when chromosome translocations occur ‐ no longer responsive to antibiotic therapy ‐ Further evolution to diffuse large B cell lymphoma ‐‐> systemic spread |
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Peripheral T‐cell Lymphoma, gen
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• More aggressive, less curable than B cell types
• TdT negative; CD2, CD5, CD3 positive; either CD4 or CD8 ‐a ß or gamma delta receptor rearrangements |
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Anaplastic Large Cell Lymphoma
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• Cytotoxic T cell lymphoma (CD8; CD2, CD3)
• Morphology: p gy large cells with horseshoe shaped nuclei and abundant cytoplasm • ALK gene rearrangement activates tyrosine kinase of JAK/STAT • Children, young adults, good prognosis |
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Adult T‐cell leukemia/lymphoma
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• Neoplasm of CD4+ T cells
• Associated with HTLV‐1 infection‐ endemic in Japan; Caribbean • Clinical ‐ Neoplastic lymphocytes proliferate in: Skin, lymph nodes, liver, spleen and bone marrow ‐‐ Peripheral blood lymphocytosis ‐ Hypercalcemia, demyelination ‐ Fatal in months to 1 yr.; no effective therapy |
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Mycosis Fungoides/Sezary Syndrome
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CD4+ helper T cell neoplasia
Slowly progressive skin involvement - rash to plaques to ulcerations and tumors. spreads to lymph and bone marrow ‐ Pautrier microabscess of mycosis fungoides -‐ Sezary cells: neoplastic CD4+ positive large, immature lymphoid cells with cerebriform nuclei |
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Large Granular Lymphocytic Leukemia
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• CD8+ T cell (or NK cell) adult leukemia
• Lymphocytosis of cells with abundant blue cytoplasm & granules • Anemia, neutropenia, splenomegaly - Felty synd, add RA Tx- chemo and steroids |
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Extranodal NK/T cell lymphoma
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NHL in Asia
nasopharynx in midline; skin; testes ischemic necrosis of vessels due to tumor surrounding them Some cases related to EBV aggressive; poorly responsive to therapy |
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Hodgkin Lymphoma (HL)
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single node
or chain of nodes • Defined by Reed‐Sternberg cells ‐Bimodal distribution |
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Pathogenisis of HL
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mutation of germinal center B-cells into Reed Sternberg cells. RS cells produce cytokines that cause infiltration and fibrosis
EBV implicated in some |
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clinical HL
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• Cervical node enlargement: 60‐80%
“horse collar” ring of enlarged nodes • Axillary nodes: 5‐20% • Inguinal nodes: 5‐12% • Mediastinal lymph node enlargement (cough, dyspnea) • Generalized pruritis • Bone pain indicates bone marrow involvement ‐ poor prognosis • Cutaneous anergy (depressed T cell function) present in most cases |
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Staging of HL
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A- no sympt B- c sympt
B= Fever, night sweats, loss of 10% body weight • Stage I: Single node region • Stage II: Two or more lymph node regions on same side of diaphragm (c limited organ involvement IIe) • Stage III: Disease on both sides of diaphagm (c spleen = IIIs) • Stage IV: Multifocal involvement of extra‐ lymphatic organs |
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for subtypes of HL
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see 13-8 on pic printout
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Treatment and prognosis of HL
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• Combination chemotherapy‐ Currently favored
• Prognosis ‐ Stage I, IIA: 90% disease‐free 5 year survival ‐ Stage IVA, IVB: 60‐70% 5 year survival |
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Thymic Hyperplasia
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• Associated with autoimmune disease:
‐ Myasthenia gravis ‐‐present in up to 75% of cases ‐ Graves disease (autoimmune thyroid disease) ‐ Collagen vascular diseases: scleroderma, SLE, RA • Morphology: lymph follicles (B cells |
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Thymoma
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• thymic epithelial cell tumors
‐ occur in adults; > 40 ‐ location: anterior superior mediastinum ‐ Benign thymoma ‐ Malignant thymoma ‐‐ Local invasion is diagnostic ‐‐ Thymic carcinoma malignant squamous histology |
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clinical thymoma
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asympt or cause pressure
Many assoc c Myasthenia Gravis sometimes c RBC aplasia or other paraneoplasia |
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Posterior mediastinum tumors
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‐ Neurogenic tumors: schwannoma, neurofibroma
‐ Lymphoma ‐ Gastroenteric hernia |
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Middle mediastinum tumors
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‐ Bronchogenic cyst; lung sequestration
‐ Pericardial cyst ‐ Lymphoma |
