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

HISTOLOGY HISTOLOGY

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

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

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

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

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.

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

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

CYTO MEGALIC VIRUS

Toxoplasma gondii

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

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

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

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

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

PART I LYMPHADENOPATHY

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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)

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

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