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Case presentation

Hala Fekry EL-Hadary Rheumatology & Rehabilitation MD Cairo University 2012

Case history

A 32 years old female presented to our clinic in May 2012 complaining of generalized bony pain and inability to walk for the last 2 years (wheel chair bound ). 12 years ago she had thrombocytopenia which improved with steroid treatment & splenectomy. She has been receiving steroids for the last 12 years in the form of prednisone 60mg/d for 10 years which was decreased gradually to 10mg/d over the last 2 years.

She hasnt been receiving any anti-osteoporotic treatment.


No other significant symptoms on history taking.

Case examination
Loss of height Severe kyphosis Protuberant chest Tender fibromyalgia points Cushinoid features

Labs Revealed:
ESR : 32 mm/hr CRP : negative HB : 8.7 g/dl WBC: 14.2/cumm PLT : 100,000/cmm Creat: 0.8 mg/dl AST: 56 IU/L ALT: 74 IU/L S.alb: 3.4g/dl Alk phos : 382 IU/L TP : 8.8 g/dl Thyroid profile: normal 10/22/2012
ANA & DNA : negative Anti-platelet Ab: positive Hepatitis C: positive Total Ca: 8.6 mg/dL S.phosp: 2.1 mg/dl PTH : 129 pg/ml 25 (OH) vit D: 15 ng/ml =
37.5nmol/L

HH

Serum 25-Hydroxyvitamin D [25(OH)D] Concentrations


nmol/L** ng/mL* Health status Associated with vitamin D deficiency, leading to rickets in infants and children and osteomalacia in adults Generally considered inadequate for bone and overall health in healthy individuals Generally considered adequate for bone and overall health in healthy individuals Emerging evidence links potential adverse effects to such high levels, particularly >150 nmol/L (>60 ng/mL)

<30

<12

3050

1220

50

20

>125

>50

1 nmol/L = 0.4 ng/mL

Radiological findings

Abd U/S : chronic parenchymetous liver disease + splenectomy + no cirrhosis

Chest x-ray :

Kyphosis + lung collapse + mild pleural effusion

X-ray Dorsolumber spine :


kyphoscoliotic

deformity of the dosal spine Multiple wedge and compression fractures of dorsal and lumbar spine 1st degree spondylolithesis of L5 over S1 Spondylotic changes are found Surgical clips are seen at left hypochondrial region ( splenectomy)

X-ray of pelvis and both hips:


Bilateral

coxa vena deformity Bones appear porotic Sub-chondral sclerotic areas are seen at the left femoral head ( ? Avascular necrosis)

DEXA
Site T-Score

Spine

6.3

Femur

5.5

Radius

6.5

Tec Bone scan :


Revealed features of metabolic bone disease causing a metabolic superascan pattern that may be attributed to either BONE MARROW DISORDER (myelo-fibrosis or myelo-sclerosis) or OSTEOMALACIA .

Tec Bone scan :


Presence of old healed fractures at the proximal third of the left tibial shaft ,in addition old mal-united fractures causing evident angulations of both humeral shafts are seen

Bone marrow aspiration


Bone marrow aspiration was done in 3/2001 Site : sternal Revealed normal bone marrow apart from hypercellularity

Key questions to this case:


Are any further investigations
necessary? Which is the correct therapeutic approach to this case ? Is it appropriate to initiate physiotherapy? Is there is a role for surgical interference? Follow up : when & what to do ?
10/22/2012 HH

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Management :

Azathiaprine :
100mg/d Prednisone : 7.5mg/d Paracetamol

Parathormone inj/d Calcium 500mg /twice Vit D : 0.25 mcg / d Nasal calcitonin 200 IU/d

10/22/2012

HH

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Patients question was :

Would I be able to walk again & when?

10/22/2012

HH

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Thanks