My Case(case presentation LCH, in SMSB

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My Case(case presentation LCH, in SMSB

  1. 1. Dr.Gaafer Ibn Auf Specialized Children's Hospital CASE PRESENTATION Dr.Ibrahim Gamer eldawla Unit Pr esent ed by: Dr. Yasser Mohammed Ahmed
  2. 2.  N e am : H .A .K .  Age : 2½ yr s.  Gender : Fem e. al  R dence esi : N H fa . ew al  Tr i be : shukr i a.  I nf or mant : her m her . ot  A i t t ed on : 31/m dm ay/2009.
  3. 3. c/o  Ski n r ash 1 yr .  Cough 1 week.
  4. 4. H.P.I  Si nce 1yr m pt . suf f er i ng f r om y ski n r ash m nl y i n t he scal p, ai f ace ant er i or chest , abdom en and upper par t of t he back. i t ’s er yt hem us m at acul o- papul ar , dr y. W hen sl oughed became hypo pi gm ed ar eas & r ed cr ust s. ent
  5. 5.  A ssoci at ed w t h m l d i t chi ng& on i i and of f l ow gr ade f ever , pt . seen i n hal f a hosp. and gi ven t opi cal t r eat ment sever al t i mes f or hi s ski n l esi ons w t h out i m ovem . i pr ent
  6. 6.  A so pt . adm t t ed t o H hospi t al l i . t hr ee t i mes dur i ng l ast f ew m hs & ever y t i m pt . need ont e m t i pl e bl ood t r ansf usi ons and ul di schar ge w t h out i def i ni t e di agnosi s.
  7. 7.  7 days PTA pt . devel oped dr y cough , SO & m l d L.L sw l i ng . B i el  Pt . di agnosed anaem c hear t f ai l ur e i + chest i nf ect i on, gi ven bl ood & ant i bi ot i cs.
  8. 8.  t he m her not i ce t hat her chi l d ot not gai ni ng W and becam t e i r r i t abl e, her appet i t e poor , t her e w abd. di st ensi on w t h nor m as i al bow habi t s. el  Then pt . r ef er r ed t o G bn auf.i hospi t al f or m e i nvest i gat i ons. or
  9. 9. Review of the systems:  MSS: t her e i s m uscl e w i ng, no ast j oi nt pai n or j oi nt sw l i ng. el  CNS: no l oss of consci ousness, no convul si ons, nor m hear i ng & vi si on al no w eakness.  Renal S : ur i ne of nor m col our , al am ount & f r eq.
  10. 10. Past.M.H. :  Past H of ear s di schar ge 3 m PTAo ext ended f or 1 m t r eat ed w t h ear o i dr yness & A . B  P H of r epeat ed hospi t al adm ssi ons & bl ood t r ansf usi ons. i  no P H of bl eedi ng .
  11. 11. Developmental History:  M pt out com of N y e SVD af t er uncom i cat ed pr egnancy. pl  N per i or post nat al pr obl em o s.  She passed t hr ough nor m m l e al i st ones t i l l age of 1 yr w hen st ar t ed t o w k w t h out suppor t al i t hen st opped her e. now j ust st and, say f ew w d, but know a or l ot .  her devel opm alent age b/w ( 1- 1.5) yr .
  12. 12. Vaccination : Vacci nat ed accor di ng t o ol d EPI w t h i B G scar pr esent . C
  13. 13. Nutritional History : Excl usi vel y br east f ed t i l l 4 mo. W eaned at 1.5 yr s. N ow on or di nar y f am l y di et i , suf f i ci ent i n (C O pr ot ei ns H, &f at s).
  14. 14. Family history: 31y 38 yr s H/W f ar mer 17y 12y 8y 5y 2.5y no FH of si m l ar condi t i on. i she has 4 si st er s, al l w e al i ve & er w l. el
  15. 15. Social History :  The l evel of educat i on of t he f at her & m her i s pr i m y school . ot ar  They l i ves i n t her e ow house w t h n i l i m t ed f aci l i t i es. i  They ar e of l ow soci o-ecnom c i st at us.  D sease has negat i ve r ef l ect i ons on i f am l y soci al l y & econom cal l y. i i
  16. 16. Drug History :  O r egul ar use of t opi cal oi nt m s n ent f or l ast one year .  not know t o be al l er gi c t o any n dr ug t aken.
  17. 17. On Examination : Pt l ooks i l l , pal e not j aundi ce not cyanosed Vi t al si gns: PR : 110 b/m ê nor m f eat ur es. al R : 35 c/m R B 85/50 nor m P: al Tem 37.6 c˚ p:
  18. 18. Anthropometric measures: W : t 8 kg bel ow t he 3r d cent i l e  Lengt h : 73cm bel ow t he 3r d cent i l e  H : 45cm .C at t he 10t h cent i l e
  19. 19.  Head& neck:  no dysm phi sm or .(00)  bot h f ont anel s w e cl osed. er  N ear s di schar ge w t h i nt act o i t ympani c m br ane. em
  20. 20.  Head& neck cont. : Lt sub m andi bul er LN pal pabl e(3 4)cm (01) firm, not fixed or tender ê nor m cover i ng al ski n. R . pr eaur i cul ar & sub m t andi bul ar LN w e er si gni f i cant l y pal pabl e (2a, 2b).  N ot her si gni f i cant l y pal pabl e o gr oup.  Exam nat i on of t he or al cavi t y& i t hr oat w nor m . as al
  21. 21.  Chest : sl i ght l y di st r essed, t r achea i s cent r al , no m ast i nal edi shi f t , nor m ai r ent r y, nor m al al vesi cul ar br eat hi ng w t h f ew i scat t er ed cr eps bi l at er al l y no w heezes.  CVS : apex at 5t h I C S j ust out M L, t her e w gal l op r hyt hm & C as shor t syst ol i c m m & LL oedem ur ur a
  22. 22.  ABDOMEN: di st ended, um l i cus f l at , bi l i ver 6 cm B M sof t , t ender , sm h C oot sur f ace, l i ver span 9 cm . spl een 4cm B M sm h sur f ace ,f i r m C , oot . ki dneys not pal pabl e, no asci t es. no pal pabl e Par a A LN her neal , or i f i ces w e, nor m ext . geni t al i a , er al PR not done.  CNS: nor m al  MMS: no j oi nt sw l i ng or el t ender ness.
  23. 23.  SKIN: m acul o papul ar br ow t o r ed r ash n cover ed al l f ace & scal p (03), t he ant er i or chest , abdomen (4a, 4b) and upper par t of t he back. upper &l ow l i m w e f r ee, er b er som r ashes heal ed t o hypo e pi gm ed ent spot s (05).
  24. 24. SKIN cont. : hai r spar se but of nor m col our . al t her e i s pet echi al r ash i n t he sol e s of f eet (06).
  25. 25. Summary  2½ yr s f em e w t h hi st or y of m al i acul o papul ar er yt hm ous ski n r ash f or one at yr , al so she had hi st or y of ot i t i s m a & f r equent B . acut el y edi T pr esent ed w t h s/s of anaem c H i i F. O/E pal e, di f f use ski n r ash. A sol t her e hepat o- spl enom egal y& cer vi cal L. adenopat hy LL edem ++. a
  26. 26. Differential diagnosis  Langer hans cel l hi st i ocyt osi s(LC ). H A LL.  Lym phom a.  Li pi d st or age di seases: –G usher 's di sease t ype1. – N em i ann Pi ck di sease t ype B .
  27. 27. Investigations: BFFM : - ve U i ne anal ysi s: cl ear . r St ool anal ysi s: cl ear . ur i ne f or m abol i c scr eeni ng : et - ve RG B: 6.1 m ol /l . m scr eeni ng f or H V: -ve I scr eeni ng f or hepat i t i s B C : -ve & m oux t est : -ve ant
  28. 28. CBC date 2/6/09 9/6/09 15/6/09 N.V HB 2 g/dl 3.4 g/dl 3.6 g/dl (11-17)g/dl RBC 660 10³/µl 1260 10³/µl 1410 10³/µl (3900-5300) 10³/µl HCT 7% 13% 11.3% (35-45)% MCV 100 fl 83 fl (80- 96) fl MCH 30 pg 25.5 pg (28- 32) pg MCHC 29 g/dl 30.5 g/dl (32- 36) g/dl TWBC 7.1 10³/µl 3.2 10³/µl 13.7 10³/µl (4.0-11.0) 10³/µl N 67 % 30 % (50-80)% L 30 % 64 % (25-50)% M 3% 6% (2-7)% Retics 6.9% (0.2-2)% PLT 64 10³/µl 30 10³/µl 34 10³/µl (150-400) 10³/µl ESR 100
  29. 29.  P.B ct ur e: .Pi Ver y sever e anaem a w t h pol y i i chr om a. asi PLT l ow . W C nor m . B al H el ect r ophor esi s: B A/A PT : 13 sec (11- 15) PTT: 26 sec (26- 36)
  30. 30. RFT & Electrolytes: Date 2/6/09 N.V urea 11 mmol/l (4- 8) mmol/l creatinine 70.4 µmol/l (70-133) µmol/l S.Sodium 130 mmol/l (132-142) mmol/l S.Potasium 3.2 mmol/l (3.2- 5.2) mmol/l S.Calcium (total) 2 mmol/l (2.1- 2.5) mmol/l S.Phosphorus 0.96 mmol/l (1.2-2.2) mmol/l S.Uric acid 202 µmol/l (100-350) µmol/l
  31. 31. LFT& Enzymes Date 3/6/09 N.V TSB 32 µmol/l <34 µmol/l Direct 11 µmol/l <3.4 µmol/l T.Protein 62 g/l (61- 75) g/l S.Albumin 26 g/l (32- 50) g/l AST 35 u/l (15- 55) u/l ALT 35 u/l (5- 45) u/l ALP 85 u/l (145-420) u/l
  32. 32. U/S abdomen 6/6  l i ver enl ar ged w t h nor m t ext ur e, i al por t al vei n not di l at ed &nor m al GB &bi l i ar y syst em .  Spl een: show m oder at e homogenous enl ar gem .ent  B h ki dneys : nor m , no cal cul i or ot al r el at ed m asses.  U :w l s sm h and r egul ar , no .B al oot cal cul i .  N abdom nal or pel vi c m o i asses or cyst s.  N f r ee f l ui ds col l ect i on. o
  33. 33.  C : (show XR )  Skul l X R ay: ( 1, 2 , 3)  L L l ong bones x R : (show ay ) R ol ogy r epor t : adi C : r i bs & bot h hum s i nvol vem XR our ent (l yt i c l esi ons), nor m l ungs+ m al od. car di om egal y. Skul l : m t i pl e l yt i c(punched out ) ul l esi ons, al so know as (geogr aphi cal n skul l ). LL: Lt f em & R . t i bi a i nvol vem . ur t ent Pel vi s& V. C um show no i nvol vem . ol n: ent
  34. 34. Bone marrow aspirate& biopsy:14/6 A r at i on: dr y t ap spi PB di m phi c bl ood pi ct ur e w t h t ar get P: or i cel l & nucl eat ed R C seen. B W C adequat e w t h m ocyt e not ed. B i yel PLT r educed. (Leuko-er yt hobl ast i c pi ct ur e ). (Show B sl i des 01, 02, 03). .M adequat e Tr ephi ne bi opsy t aken w t h i f r agm ed bony t r abecul ae ext r em y ent el hyper cel l uar w t h depr essed i haem opoi esi s, m r ow i s i nf i l t r at e by ar
  35. 35. Bone marrow aspirate& biopsy:14/6 f i ndi ng consi st ent w t h LC . i H f or speci al st ai n w t h C 1a, S100. i D st ai ni ng w t h S100 w i as +ve (t el ephone com ent done i n m l i t ar y m i hosp.)
  36. 36. DIAGNOSIS : LCH class IIIa.
  37. 37. Management :  Counsel i ng.  Suppor t i ve t r eat m . ent B ood t r ansf usi on l t opi cal oi nt ment C hem her apy. ot st er oi d& vi nbl ast i n  Fol l ow up.
  38. 38. Fol l ow up pl an: 1. C i ni cal l y. 0a, 0b, 0c l 2. Lab : – C Cdone 8/8/009 , LFT , bl eedi ng B pr of i l e . – B one m r ow 1a , 1b). done13/8/009 ar .( (conc: LC i n hem ol ogi cal H at r em ssi on). i 3. R adi ol ogi cal : – C & Skel et al sur vey. XR 4. EN consul t at i on. T
  39. 39. Literature review :  Histiocytic Disorders  cl ass1 ( LC ) H N m hi st i ocyt es or i gi nat e f r om or al pl eur i pot ent st em cel l s . Under t he ef f ect of var i ous cyt oki nes , hi st i ocyt es di f f er ent i at e t o speci al i zed cel l s :m onocyt es ,t i ssue m ophages acr dendr i t i c cel l s and l anger hans cel l s. t hese cel l s becam ant i gen pr esent i ng e cel l s and som have phagocyt i c e act i vi t i es.
  40. 40.  H st i ocyt osi s ar e het er ogeneous gr oup i of uncom on pr ol i f er at i ve di seases m i nvol vi ng B der i ved i m at ur e .M m hi st i ocyt i c cel l s , w ch can have m e hi or r eact i ve t han m i gnant f eat ur es. al  W O cl assi f i cat i on of hi st i ocyt i c H di sor der s:  C ass I l dendr i t i c cel l r el at ed di sor der s. (LC ) : H I Si ngl e bone II M t i pl e bone ul  I I I A bone + sof t t i ssues
  41. 41.  C ass I I (m ophage r el at ed di sor der ) l acr : 1. H st i ocyt osi s of m i ononucl ear phagocyt es ot her t han LC s 2. 1r y& 2r y hem ophagocyt i c l ym phohi st i ocyt osi s. 3. Si nus hi st i ocyt osi s w t h m i assi ve l ym phadenopat hy (R osai -D f m or an) 4. Juveni l e xant hogr anul om (JXG a ) 5. R i cul o hi st i ocyt om et a
  42. 42.  C ass I I I M i gnant hi st i ocyt i c l al di sor der s : 1. A cut e monocyt i c l eukem a (FA M i B 5). 2. M i gnant hi st i ocyt osi s. al 3. Tr ue hi st i ocyt i c l ymphom . a
  43. 43.  C i dence l evel s f or t he di agnosi s of onf LC :H – pr esum i ve : l i ght m phol ogi c pt or char act er i st i cs . – Designated : above + ≥2 posi t i ve st ai ns of : 1. A denosi ne t r i phosphat ase 2. S-100 pr ot ei n. 3. A pha –D m l - annosi dase . 4. Peanut l ect i n. – D i ni t i ve : l i ght + B r beck gr anul es ef i and/ or C 1aD
  44. 44. Poor pr ognost i c f eat ur es : 1. I nvol vem ent of t he r i sk or gans w t hi dysf unct i on (l ungs ,br ai n , l i ver , B ). M 2. Lack of r api d r esponse t o chem her apy ot . 3. A bsence of bone di sease  The w st pr ognosi s i s associ at ed w t h or i cl ass I I I b (Let t er er Si w i t h a e),w 5year s sur vi val of 50% w t h i nt ensi ve i chem her apy. ot  A under 2 yr s at di agnosi s w t hout ge i “R SK I ” or gan
  45. 45. THANKS

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