LYMPHADENOPATHY Dr. Manjit Singh Saren Pathologist, MAHSA University College.

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1 LYMPHADENOPATHY Dr. Manjit Singh Saren Pathologist, MAHSA University College.

2 HISTOLOGY HISTOLOGY

3 LYMPHADENOPATHY DEFINITION: Disease of lymph nodes Associated with underlying pathology. i) Involvement in variety of systemic diseases. ii) Primary lymphoid malignancies iii) Metastatic lymphoid lesions iv) Localized infections v) Localized injuries

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5 LYMPHADENOPATHY Types: 1. REACTIVE LYMPHADENITIS Inflammation of lymph nodes 2. REACTIVE LYMPHADENOPATHY Primary immune reactions

6 REACTIVE LYMPHADENITIS NON- SPECIFIC RESPONSE 1.Acute lymphadenitis 2.Chronic lymphadenitis 1) Acute lymphadenitis: draining inflamed area. >Bacterial infections >Foreign bodies in wounds Nodes: Enlarged and tender Untreated : Chronic adenitis, necrosis and abscess

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8 2 ) Chronic Lymphadenitis: A). With follicular B cell hyperplasia i.Non- specific lymphadenitis: Commonest lesion ii. Specific lymphadenitis: Rheumatoid arthritis Toxoplasmosis Syphilis AIDS

9 B) With para-follicular T cell hyperplasia - Microbiological agents - Drugs (Dilantin) - Virus (Inf. Mononucleosis) -Post vaccinial C) With sinus hyperplasia with histiocytosis: Cancer draining sites. Micro: Phagocytes within dilated sinuses.

10 LYMPHADENOPATHY 1.Primary: Lymphoid malignancies. Amyloidosis 2. Secondary: i. Infections: TB Sarcoidosis Syphilis Castleman’s Disease Dermatolymadenopathy Filariasis ii. Metastatic diseases

11 CAUSES OF LYMPADENOPATHY: 1.HIV related 2.Opportunistc infections: TB, CMV, Toxoplasmosis, Norcardia. 3.Fungal: Histoplasmosis, cryptococcusis. 4.Reactive: Pyogenic infections/bacteria. 5.Venereal: (STD)- Syphilis, Chancroid, Lympho- Granuloma Venereum 6.Malignancies: i. Primary: Lymphomas ii. Secondary: Metastasis

12 CYTO MEGALIC VIRUS

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14 Toxoplasma gondii

15 TUBERCULOUS LYMPHADENOPATHY Poor socio-economic background. Over-crowding Presentation: Night sweats Cough with hemoptysis Loss of weight Family history

16 Diagnosis History ESR Sputum examination for AFB TB Culture Chest X-rays CT Scan MRI BIOPSY

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20 SARCOIDOSIS Etiology: Unknown. Cell mediated immune reaction Affects: Lung and lymph nodes Micro: 1. Langhans cells, Foreign body giant cells 2. Asteroid bodies, Schaumann bodies. 3. Non-caseating granuloma 4. Epitheloid cells and fibrosis Diagnosis: Kviem’s Test: Intra-dermal test

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22 FILARIAL LYMPHADENOPATHY Etiology: Wuchereia Bancrofti Brugia malayi Adult worm in: lymphatics, Lymph nodes, testis and epididymis Acute: Fever, lymphangitis, epididymo-orchitis and microfilaria. Chronic: Lymphadenopathy, hydrocele and elephantiasis. Complications: Chylous ascitis & chyluria

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24 AL AMYLOIDOSIS Associated with plasma cell dyscrasias. Secrete Immunoglobulin light chains. Can be associated with lymphadenoparthy Diagnosis: Congo red: 1.Light microscopy Eosinophilic amorphous 2.Polarised light: Apple green birefringence

25 PART I LYMPHADENOPATHY

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27 CASTLEMAN’S DISEASE Definition: Single solid growth within lymphatic tissue Sites: Chest, stomach or neck. Abnormal enlargement of lymph nodes.

28 CASTLEMAN’S DISEASE Etiology: Unknown Rare disorder Types: 1. Hyaline type 90% 2. Plasma type: prevalent in young

29 DERMOTOPATHIC LYMPHADENOPATHY Definition: Reactive lymph node hyperplasia. Etiology: Secondary to eczema and dermatitis Micro: Intra-dermal macrophages containing fat and melanin

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31 CAT SCRATCH DISEASE Children 80% Self limiting Gm-ve bacterial infection Localized lymphadenopathy Sarcoid-like granulomas Stellate abscess

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33 LYMPHANGIITIS Inflammation of lymphatic vessels i. Acute Lymphangiitis: Bacterial infections: B-Strep % & Staph. Micro: inflammatory exudate and clotted lymph ii. Chronic Lymphangitis: TB, Actinomyces, syphilis, radiation,& parasites Micro: Fibrosis and chronic lymphedema

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36 KIKUCHI’S DISEASE Presentation: Fever and Tender Adenitis Micro: Necrosis and stellate abscesses Neutrophils and nuclear dust Histiocytes but rarely any eosinophils

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38 KIMURA’S DISEASE Introduction: Chronic inflammatory disorder Clinical Features: Age: 20-40 yrs and males Painless swelling of parotid Cervical lymphadenopathy

39 KIMURA’S DISEASE Micro: Lymph node biopsy: Eosinoplilia Proliferation of vascular endothelium. Focal eosinophilic abscess Hyperplasia of lymphoid follicles: Well vascularised Warthin-Finkeldey polykaryocytes

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42 KIKUCHI’S DISEASE Common in children Cervical lymphadenopathy Also : Necrotizing histiocytic lymphadenitis Etiology: CMV EBV Herpes virus

43 KIMURA’S DISEASE PATHOPHYSIOLOGY Allergic reaction: -Parasites, viral, arthropod bites and candidiasis. Auto-immune reaction : IgE –mediated Type I hypersensitive reaction. Cytokines: Interleukin 4 and 5 produces:-  > Eosinophiltrophic cytokines & IgE 20% with nephrotic syndrome

44 KAWASAKI’S DISEASE Age: 39C) red eyes Red and cracked lips Rash on chest and genitals Swollen tongue and enlarged lymph nodes Complications: Affects heart: Arrhythmias

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49 C)Multicentric or Generalized Type CASTLEMAN’S DISEASE Multiple sites Symptoms: Same as plasma type Hepato-splenomegaly ‘POEMS Syndrome Complications : POEMS SYNDROME Kaposi’s Sarcoma Non Hodgkin’s Lymphoma

50 LANGERHANS CELL HISTIOCYTOSIS Histiocytosis : Proliferative disorder of histiocytes or macrophages. Auto-immune disease & genetically related Types: 1. Benign: Histiocytic proliferation in lymph nodes. 2. Malignant: Histiocytic lymphoma 3. Intermediate: Langerhans cell Histiocytosis

51 LANGHERAN CELLS: ORIGIN Derived from dendritic cells of skin. Proliferating Langerhans cells are LEUKOCYTE ANTIGEN DR (HLA-DR) positive and express CD1 antigen. Cells contain Berkeck’s granules. EM Micro: Vacuolated cytoplasm resembling tissue histiocytes and hence called Langerhans Cell Histiocytosis

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53 HISTIOCYTOSIS X 1. LITTERER SEWE DISEASE Age: 2 yrs and less Presentation:Cutaneous lesions Hepato-splenomegaly Pulmonary lesions (stiffening) Destructive osteolytic bone lesions&fractures Infiltrates bone marrow causing: anaemia and thrombocytopenia Predisposing: to infections. Rapidly fatal Chemo:50% 5 year survival

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55 2: EOSINOPHILIC GRANULOMA Langerhans cells in bone medullary cavities Micro: Histiocytes and eosinophils along with plasma cells and lymphocytes. Lesions: Unifocal: Pain and pathological # Treatment: Local excision and radiation. May heal spontaneously

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57 2. MULTIFOCAL LESIONS Affects children Clinical Presentation: Fever and diffuse eruptions on scalp Otitis media URTI Generalized Lymphadenopathy Hepato-splenomegaly Diabetes Insipidus (post pituitary stalk involved)

58 3. HAND SCHULLER CHRISTIAN DISEASE Presentation: Involvement of calvarial bone Diabetes Insipidus Exophthalmos Treatment: Spontaneous regression Chemotherapy

59 HAND SCHULLER CHRISTIAN DISEASE Presentation: Involvement of calvarial bone Diabetes Insipidus Exophthalmos Treatment: Spontaneous regression Chemotherapy