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Cold agglutinins: Raynaud’s, cyanosis / cold T changes surfaces (affects presentation of Ag to IgM creating an acquired von Willebrand’s (effect) / can lead to infarctions
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- Angioimmunoblastic lymphadenopathy with dysproteinemia
- Non-Hodgkin’s Lymphoma
Cold agglutinins: Raynaud’s, cyanosis / cold T changes surfaces (affects presentation of Ag to IgM creating an acquired von Willebrand’s (effect) / can lead to infarctionsLungs: infiltrates (plasma cell interstitial pneumonia), isolated masses, pleural effusions Infection: double rate / PCP, CMV, HCV Amyloidosis (less common): see amyloidosis Renal failure (less common): more chronic than acute / amyloidosis, Bence- Jones proteinuria (33%), IgM deposition, IgM occlusion Labs: Coomb’s positive, cryoglobulins, hyponatremia (from dilution), hyperproteinemia (total protein and albumin), hypercalcemia, normocytic anemia (decreased RBC survival, Fe deficiency, increased plasma volume, not so much hemolytic anemia but there is some extravascular causing spherocytosis), azotemia, elevated inflammatory markers (ESR, RF, C3/C4, CPK), ?hepatitis immunity, normal WBC (but with lymphocytosis or monocytosis) Diagnosis: SPEP with monoclonal spike (IgM always elevated, IgG decreased in 60%, IgA decreased in 20%), bone marrow biopsy Treatment: plasmapheresis for immediate benefit (based on symptoms, not labs), followed by proper chemotherapy (cladribine, fludarabine and 2-deoxychloroagenosine are accepted agents) / rituximab (anti-CD20) / high-dose autologous hematopoeitic stem-cell transplantation / bisphosphonates unnecessary because no bone/lytic lesions Survival: 80% response to chemotherapy / median survival 3-5 years Schnitzler’s syndrome Urticarial vasculitis with macroglobulinemia / nodular, macular infiltration of tumor cells or pruritic papules from IgM deposition / can get urticaria and crusted, hemorrhagic lesions from type I cryoglobulinemia Heavy chain disease age 10 - 30 / heavy chain fragments, do not react with anti-lambda or anti-kappa Angioimmunoblastic lymphadenopathy with dysproteinemiaWidespread, rapidly progressive (weight loss, hepatosplenomegaly, fevers), usu. polyclonal Castleman’s Disease (angiofollicular lymph node hyperplasia) often associated with HIV/AIDS, but can occur by itself / HHV-8, EBV Hyaline vascular type fever, anemia, hypoalbuminemia (~100%), organomegaly (90%), edema, pleural/pericardial effusions or ascites (50%), rash (33%), pancytopenia (35%) Labs: polyclonal SPEP, elevated ESR Plasma cell type – only 10% / neuropathy POEMS syndrome (⅔ will have Castleman’s plasma cell type) Polyneuropathy, Organomegaly, Endocrinopathy, M protein (lambda in 80%), Skin changes / usually occurs in pts with osteosclerotic multiple myeloma who also have hepatomegaly, diabetes, gynecomastia, thickening and hyperpigmentation of the skin and sensorimotor polyneuropathy Lymphoma Hodgkin’s Disease 2 in 100,0000 / 15-35 yrs (nodular sclerosis) or over 50 yrs (mixed cellularity) Presentation: non-painful swelling of neck lymph node / fever, night sweats, weight loss, pruritis (rare) / contiguous nodal spread / erythema nodosum, icthyosis Diagnosis: biopsy: important to get excisional biopsy rather than FNA in order to see nodal architecture / must see Reed-Sternberg cell (or lacunar cell in nodular variant) on biopsy; unfortunately, RS-like cells have been found in EBV, solid cancers, fungal infections, and other staging: abdominal CT/MRI, gallium scans (sometimes), bone marrow biopsy (sometimes) Complications: suppression of DTH response (T-cell problem) Prognosis: B-symptoms, older age, stage, certain histological type give worse prognosis Treatment: radiation and/or chemotherapy (MOPP) nodular sclerosis (1st) excellent prognosis more common in women / often involves lower cervical, supraclavicular and mediastinal nodes / distinct Reed Sternberg (RS) variant known as “lacunar cell” due to retraction of cytoplasm during fixation collagen bands divide tissue into nodules [pic] / classic RS cells infrequent [pic] mixed cellularity (2nd) clinical picture in between lymphocyte predominance and depletion pattern / heterogeneous cellular infiltrate small areas of fibrosis and necrosis / many typical RS cells (binucleate with inclusion-like nucleoli w/ halo) / pts present with disseminated involvement and systemic manifestations / more common in males lymphocyte predominance (3rd) excellent prognosis young males, diffuse, sometimes vaguely nodular infiltrate of mature lymphocytes admixed with a variable number of histiocytes / typical RS cell gives way to “popcorn cell” lymphocyte depletion (4th) poor prognosis older males / disseminated involvement, systemic manifestations / aggressive diffuse fibrosis - proteinaceous fibrillar material w/ some RS cells and lymphocytes reticular variant - highly anaplastic, large, pleomorphic cells / limited number of typical RS cells Non-Hodgkin’s Lymphomaover 50 yrs / disseminated (childhood intraabdominal, CNS, bone) / B-cell malignancy (80%) / incidence increased dramatically in US in last 50 years / HIV increases risk Presentation: GI complaints, weight loss, neuropathy (usually focal or oligo in distribution; CNS involvement at presentation occurs in 2%), may have hemolytic anemia (warm antibodies/look for spherocytes), thrombocytopenia Pathology: nodal architecture, degree of differentiation, cell origin Diagnosis: imaging studies, testicular ultrasound, cytologic analysis / in CSF, high false negative
low grade / diffuse nodal architecture / involves bone marrow, peripheral blood, spleen / CLL Follicular small cleaved cell lymphoma low grade / “cleaved” nuclear contour / bone marrow / t (14:18), bcl-2 Diffuse large cell lymphoma (DLBCL) high grade B-cell / increased risk in AIDS patients Prognosis: international prognostic index (IPI) / age, LDH, performance status, Ann Arbor stage, > 1 node involved [cure rate from 20-70% depending on score] Treatment: chemotherapy or BMT (being studied) Primary cutaneous large cell lymphoma (recently described) Cd30+ / negative for 2:5 translocation and ALK expression Differential: lymphomatoid papulosis (small papules that regress spontaneously), primary cut B-cell, SALT lymphomas (recently recognized), low-grade, EORTC Lymphomatoid granulomatosis [NEJM] large B-cell lymphoma / CD-20 positive / EBV association Pulmonary: multiple bilateral nodules (usu. not mediastinal and hilar) Skin: various manifestations (maculopapular, subcutaneous nodules), usu. not confluent [pic] CNS: mass or isolated cranial neuropathies Ddx: lymphoma, LIP, metastatic cancer, sarcoidosis, Wegener’s, cryptogenic organizing pneumonia Treatment: combination chemotherapy (steroids, CTX, rituximab, IFN-alpha) Small non-cleaved cell lymphoma (Burkitt’s lymphoma) EBV / t(8:14) w/ c-myc / jaw mass (Africa), GI, gonad (US) / large lymphoid cells w/ dark blue cytoplasm (Wright stain) / “starry sky” appearance are benign macrophages interspersed ALL L3 vacuoles [pic][pic] / different form in AIDS patients Marginal zone B-cell lymphoma CD20+, CD5 (-) / extranodal are considered as MALToma MALT lymphoma (MALToma) B- cell / more localized to primary site / stomach (H. pylori) > orbit, adrenal (C. psittaci), intestine (C. jejuni), lung, thyroid, salivary gland, skin (Borrelia), soft tissue, bladder, kidney, CNS) / t(11:12) is worse / treat with chemotherapy / associated with autoimmune disease or chronic inflammation / in case of H. pylori gastric MALToma, may actually regress with eradication of H. pylori T-cell lymphomas Adult T-cell leukemia – poor prognosis Presentation: skin lesions, lymphadenopathy, hepatosplenomegaly / often confused for sarcoidosis / may present as pneumonia [CXR] Epidemiology: caused by HTLV-1 (virus) / lifetime risk in carriers 2.6% in women, 4.5% in men / high incidence in SW Japan, Caribbean, Northern South America Diagnosis: hypercalcemia, elevated WBC, multilobated, atypical lymphocytes [pic] Tissue biopsy: must have lab look for chromosomal rearrangement Course: aggressive growth / ~8 month survival Lymphoblastic lymphoma males 2:1 / T-cell ALL / < 20 yrs / 40% of childhood lymphomas / mediastinal mass high cure rate, but frequent CNS relapse from earlier seeding
Most respond, relapse is the rule Angiocentric nasal T/natural killer (NK) cell lymphoma southern Asia and Latin America Lymphoma in HIV/AIDS patients 6% risk, increases over time (unlike Kaposi’s) Treatment: low-dose modified chemotherapy under investigation / average survival 2 to 4 months 60% grade III/IV – older patients, later stage of AIDS / 90% B-cell / over ½ with EBV DNA / 20% Burkitt’s lymphoma – younger patients, earlier stage of AIDS 20% Primary CNS lymphoma Diagnosis: EBV DNA PCR positive in 90% of cases / stereotactic brain biopsy Presentation: focal neurologic signs, cranial nerve deficits, headaches, seizures / 1 to 3 lesions (3 to 5 cm, often multiple, ring enhancing lesions) / leptomeningeal involvement (40%; 8% as sole manifestation) Treatment: radiation therapy Systemic lymphomas in AIDS GI (25%)
Bone marrow (20%) Liver (10%) Lungs (10%) – discrete or interstitial CNS (20%) – must do LP to stage any systemic lymphoma with AIDS Bone [orthopedics] Bone Malformations Bone Cancer Osteoporosis General Fluoride concentrations should exceed 1 or 10? ppm in water supply Supplementation for exclusively breast fed infants, certain types of formula Note: increased pressure in bone causes bone pain Orthopedics Knee Pain, Low Back Pain, Bone Fractures, Bone Malformations Download 3.95 Mb. Share with your friends:
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