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LGH

LGH. History. 48 y female seen in OPD 2 Months SOBE , Dry cough Wt loss & Fatigue No orthopnea , PND , Chest pain , wheeze No hemoptysis , fever , night sweating. History. Able to walk 2 blocks No nausea , vomiting , abdominal pain

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LGH

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  1. LGH

  2. History • 48 y female seen in OPD • 2 Months SOBE , Dry cough Wt loss & Fatigue • No orthopnea , PND , Chest pain , wheeze • No hemoptysis , fever , night sweating

  3. History • Able to walk 2 blocks • No nausea , vomiting , abdominal pain • No joint pain , swelling or skin rash • No contact with sick person

  4. History • Smoker 30 pack • PMH : NHL stage IB 1998 cervical LN Chemo & Radiotherapy 1999 In remission followed in cancer care Previous IVDU  -ve HIV , HBV & HCV No previous pneuomnia • No pets , occupational exposure

  5. History • No medication , travel • PSH : tonsilectomy ,tubal ligation • FH unremarkable • Referred by radiation oncology because of symptoms & abnormal CXR

  6. Examination • Afebrile RR 14 Sat 95% RA BP 160/70 HR 90 • No Lymphadenopathy , clubbing • Chest : Good breath sound No wheeze , crackles

  7. Examination • CVS : S1+S2+0 • Abd : No splenomegaly • No LL edema • No skin rash , joint swelling

  8. Investigation • CBC  Hb 110 MCV N • Coagulation N • BUN , Creat & Electrolyte N • LFT & LDH N • CXR , Chest CT & PFT

  9. Work Up • ANA , ANCA , RF & complement N • 6MWT distance 480 m Sat 95%  89% HR 70 100 • BAL  -ve culture & cytology • Transbronchial Bx  non specific

  10. Work Up • Open lung Bx Langerhans Cell Histiocytosis

  11. Langerhans Cell Histiocytosis • Langerhans cell is an antigen presenting cell • Origin monocyte – macrophage cell linage • Normally found in dermis, reticuloendothelial system, lung, & pleura.

  12. Langerhans Cell Histiocytosis • Can be found in association with cigarette smoking in asymptomatic individuals and in other pulmonary disorders, such as IPF • LCH is a spectrum of diseases caused by proliferation & infiltration of LG cells • First described early 1950s

  13. Langerhans Cell Histiocytosis • Different presentations with confusing classification. • No genetic , geographic or occupational predisposition • 2 unique pathological features Birbeck granules by EM (intracellular structure) CD1a antigen on cell surface Protein s 100

  14. Pulmonary LCH • Unknown true incidence or prevalence • 5% of open lung Bx for ILD work up ? Underestimation • Disease of Caucasian , Smokers • Young age with equal M:F {? Because of increasing smoking in females}

  15. Presentations • IncidentalCXR finding • Fatigue ,wt loss • Respiratory : 6 – 12 months SOBE , dry cough , rib pain pneumothorax • Extrapulmonary : DI ,bone or skin lesions

  16. Presentations • Clinical exam : unremarkable Core pulmonale in advanced cases Bony or skin lesions • Laboratory : Non specific DI picture in serum & urine electrolytes & osmolality

  17. Diagnosis • Radiological: bilateral symmetrical disease Ill-defined or stellate nodules (2 to 10 mm in size)    Reticulonodular infiltrates    Upper zone cysts or honeycombing    Preservation of lung volume    Costophrenic angle sparing ( different than LAM )

  18. Diagnosis • Radiological: Correlation between radiological & pathological progression Cellular infiltrate { Nodules } around small airways cavitations replacement by fibroblast {satellite lesions & Cyst formation } Simultaneous presence of nodules & cyst is highly suggestive of PLCH

  19. Diagnosis • Radiological: DDx  LAM , Tuberous sclerosis Hypersensitivity pneumonitis Sarciodosis , IPF Mycobacterial & Fungal infections Malignancy , Wegner vasculitis

  20. Diagnosis • PFT & CPET: Variable , sometimes out of proportion to radiological finding Obstructive out of proportion to smoking , unique to PLCH & LAM than other ILD Restrictive or mixed Limited CPET > COPD due presence of primary pulmonary vasculopathy

  21. Diagnosis • BAL: Low yeild10-40%  focal disease DC1a stained cells in BAL > 5% had a good sensitivity ( single study & small number ) • Open Lung Bx Gold standard In symptomatic pateints , equivocal radiological finding & possibility of another Dx cant be excluded

  22. Treatment • No RCT • Smoking cessation associated with slow progression • Steroid & chemo has been used in few cases • Transplantation in young who quit smoking with rapid progression ?Recurrence after transplant

  23. Prognosis • Variable • Poor prognostic factors : extreme of ages diffuse progressive disease multisystem involvement • Risk Of lung Ca : ? pure smoking related risk Vs PLCH additive risk

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