LGHLGH

HistoryHistory

48 y female seen in OPD48 y female seen in OPD

2 Months SOBE , Dry cough2 Months SOBE , Dry cough Wt loss & FatigueWt loss & Fatigue

No orthopnea , PND , Chest pain , wheezeNo orthopnea , PND , Chest pain , wheeze

No hemoptysis , fever , night sweating No hemoptysis , fever , night sweating

HistoryHistory

Able to walk 2 blocksAble to walk 2 blocks

No nausea , vomiting , abdominal painNo nausea , vomiting , abdominal pain

No joint pain , swelling or skin rashNo joint pain , swelling or skin rash

No contact with sick personNo contact with sick person

HistoryHistory Smoker 30 pack Smoker 30 pack

PMH : NHL stage IB 1998 cervical LNPMH : NHL stage IB 1998 cervical LN Chemo & Radiotherapy 1999Chemo & Radiotherapy 1999 In remission followed in cancer careIn remission followed in cancer carePrevious IVDU Previous IVDU -ve HIV , HBV & HCV -ve HIV , HBV & HCVNo previous pneuomniaNo previous pneuomnia

No pets , occupational exposureNo pets , occupational exposure

HistoryHistory No medication , travelNo medication , travel

PSH : tonsilectomy ,tubal ligationPSH : tonsilectomy ,tubal ligation

FH unremarkableFH unremarkable

Referred by radiation oncology because of Referred by radiation oncology because of symptoms & abnormal CXRsymptoms & abnormal CXR

ExaminationExamination

Afebrile RR 14 Sat 95% RAAfebrile RR 14 Sat 95% RA

BP 160/70 HR 90BP 160/70 HR 90

No Lymphadenopathy , clubbingNo Lymphadenopathy , clubbing

Chest : Good breath sound Chest : Good breath sound

No wheeze , cracklesNo wheeze , crackles

ExaminationExamination

CVS : S1+S2+0CVS : S1+S2+0

Abd : No splenomegalyAbd : No splenomegaly

No LL edemaNo LL edema

No skin rash , joint swellingNo skin rash , joint swelling

InvestigationInvestigation

CBC CBC Hb 110 MCV N Hb 110 MCV N

Coagulation NCoagulation N

BUN , Creat & Electrolyte NBUN , Creat & Electrolyte N

LFT & LDH NLFT & LDH N

CXR , Chest CT & PFT CXR , Chest CT & PFT

Work UpWork Up ANA , ANCA , RF & complement NANA , ANCA , RF & complement N

6MWT distance 480 m 6MWT distance 480 m

Sat 95% Sat 95% 89% 89%

HR 70 HR 70 100100 BAL BAL -ve culture & cytology -ve culture & cytology

Transbronchial Bx Transbronchial Bx non specific non specific

Work UpWork Up

Open lung BxOpen lung Bx

Langerhans Cell HistiocytosisLangerhans Cell Histiocytosis

Langerhans Cell HistiocytosisLangerhans Cell Histiocytosis

Langerhans cell is an antigen presenting cellLangerhans cell is an antigen presenting cell

Origin Origin monocyte – macrophage cell linagemonocyte – macrophage cell linage

Normally found in dermis, reticuloendothelial Normally found in dermis, reticuloendothelial system, lung, & pleura.system, lung, & pleura.

Langerhans Cell HistiocytosisLangerhans Cell Histiocytosis

Can be found in association with cigarette Can be found in association with cigarette smoking in asymptomatic individuals and in smoking in asymptomatic individuals and in other pulmonary disorders, such as IPFother pulmonary disorders, such as IPF

LCH is a spectrum of diseases caused by LCH is a spectrum of diseases caused by proliferation & infiltration of LG cellsproliferation & infiltration of LG cells

First described early 1950s First described early 1950s

Langerhans Cell HistiocytosisLangerhans Cell Histiocytosis

Different presentations with confusing Different presentations with confusing classification.classification.

No genetic , geographic or occupational No genetic , geographic or occupational predisposition predisposition

2 unique pathological features2 unique pathological features

Birbeck granules by EM (intracellular structure)Birbeck granules by EM (intracellular structure)

CD1a antigen on cell surface Protein s 100CD1a antigen on cell surface Protein s 100

Pulmonary LCHPulmonary LCH Unknown true incidence or prevalenceUnknown true incidence or prevalence

5% of open lung Bx for ILD work up 5% of open lung Bx for ILD work up ? Underestimation ? Underestimation

Disease of Caucasian , SmokersDisease of Caucasian , Smokers

Young age with equal M:F Young age with equal M:F {? Because of increasing smoking in females}{? Because of increasing smoking in females}

PresentationsPresentations IncidentalIncidental CXR findingCXR finding

Fatigue ,wt loss Fatigue ,wt loss

Respiratory : 6 – 12 monthsRespiratory : 6 – 12 months SOBE , dry cough , rib painSOBE , dry cough , rib pain pneumothoraxpneumothorax

Extrapulmonary : DI ,bone or skin lesionsExtrapulmonary : DI ,bone or skin lesions

PresentationsPresentations Clinical exam :Clinical exam : unremarkable unremarkable

Core pulmonale in advanced casesCore pulmonale in advanced cases

Bony or skin lesionsBony or skin lesions

Laboratory :Laboratory : Non specificNon specific

DI picture in serum & urine electrolytes DI picture in serum & urine electrolytes

& osmolality& osmolality

DiagnosisDiagnosis Radiological: Radiological: bilateral symmetrical diseasebilateral symmetrical disease

Ill-defined or stellate nodules (2 to 10 mm in size)Ill-defined or stellate nodules (2 to 10 mm in size)

       Reticulonodular infiltratesReticulonodular infiltrates

    Upper zone cysts or honeycombing    Upper zone cysts or honeycombing

    Preservation of lung volume    Preservation of lung volume

    Costophrenic angle sparing ( different than LAM )    Costophrenic angle sparing ( different than LAM )

DiagnosisDiagnosis

Radiological:Radiological:

Correlation between radiological & pathological Correlation between radiological & pathological progressionprogression

Cellular infiltrate { Nodules } around small Cellular infiltrate { Nodules } around small airways airways cavitations cavitations replacement by replacement by fibroblast {satellite lesions & Cyst formation }fibroblast {satellite lesions & Cyst formation }

Simultaneous presence of nodules & cyst is Simultaneous presence of nodules & cyst is highly suggestive of PLCHhighly suggestive of PLCH

DiagnosisDiagnosis

Radiological:Radiological:DDx DDx LAM , LAM ,

Tuberous sclerosisTuberous sclerosis

Hypersensitivity pneumonitisHypersensitivity pneumonitis

Sarciodosis , IPFSarciodosis , IPF

Mycobacterial & Fungal infectionsMycobacterial & Fungal infections

Malignancy , Wegner vasculitisMalignancy , Wegner vasculitis

DiagnosisDiagnosis PFT & CPET: PFT & CPET: Variable , sometimes out of proportion to Variable , sometimes out of proportion to

radiological findingradiological findingObstructive out of proportion to smoking , Obstructive out of proportion to smoking ,

unique to PLCH & LAM than other ILD unique to PLCH & LAM than other ILD Restrictive or mixed Restrictive or mixed

Limited CPET > COPD due presence of Limited CPET > COPD due presence of primary pulmonary vasculopathy primary pulmonary vasculopathy

DiagnosisDiagnosis BAL:BAL:

Low yeildLow yeild 10-40% 10-40% focal disease focal disease

DC1a stained cells in BAL > 5% had a good sensitivity DC1a stained cells in BAL > 5% had a good sensitivity ( single study & small number )( single study & small number )

Open Lung BxOpen Lung BxGold standard Gold standard

In symptomatic pateints , equivocal radiological finding In symptomatic pateints , equivocal radiological finding

& possibility of another Dx cant be excluded & possibility of another Dx cant be excluded

TreatmentTreatment No RCT No RCT

Smoking cessation associated with slow Smoking cessation associated with slow progressionprogression

Steroid & chemo has been used in few casesSteroid & chemo has been used in few cases

Transplantation Transplantation in young who quit smoking in young who quit smoking with rapid progression with rapid progression ?Recurrence after transplant ?Recurrence after transplant

PrognosisPrognosis

VariableVariable

Poor prognostic factors : Poor prognostic factors :

extreme of ages extreme of ages

diffuse progressive diseasediffuse progressive disease

multisystem involvement multisystem involvement

Risk Of lung Ca : Risk Of lung Ca :

? pure smoking related risk Vs PLCH additive risk? pure smoking related risk Vs PLCH additive risk