Indan Journal of Medical Research Indan Journal of Medical Research Indan Journal of Medical Research Indan Journal of Medical Research
  Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login  
  Home Print this page Email this page Small font sizeDefault font sizeIncrease font size Users Online: 373       

   Table of Contents      
CLINICAL IMAGES
Year : 2015  |  Volume : 141  |  Issue : 2  |  Page : 249-250

Hughes syndrome with cerebral, skeletal infarction & retinal vein thrombosis


Department of Nuclear Medicine & PET CT, Amrita Institute of Medical Sciences, Amrita Vishwavidyapeetham University, Cochin 682 041, Kerala, India

Date of Web Publication21-Apr-2015

Correspondence Address:
P Shanmuga Sundaram
Department of Nuclear Medicine & PET CT, Amrita Institute of Medical Sciences, Amrita Vishwavidyapeetham University, Cochin 682 041, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-5916.155600

Rights and Permissions

How to cite this article:
Sundaram P S, Padma S. Hughes syndrome with cerebral, skeletal infarction & retinal vein thrombosis. Indian J Med Res 2015;141:249-50

How to cite this URL:
Sundaram P S, Padma S. Hughes syndrome with cerebral, skeletal infarction & retinal vein thrombosis. Indian J Med Res [serial online] 2015 [cited 2019 Nov 20];141:249-50. Available from: http://www.ijmr.org.in/text.asp?2015/141/2/249/155600

A 23-year old female patient presented to the General Medicine and Nuclear Medicine departments of Amrita Institute of Medical Sciences, Cochin, Kerala, India in March 2013 with fever, joint pains for six months duration with thrombocytopenia. Immunologically she was tested positive for systemic lupus erythematosis (SLE). A Technetium 99m (Tc 99m)-methylene diphosphonate (MDP) bone scan was advised. Images revealed unsuspected cold defects in D6 and L4 vertebral bodies raising the suspicion of bone infarcts versus marrow metastatic infiltration. The patient showed no evidence of polyarthritis. However, there was evidence for left frontal lobe acute infarction on the MRI (magnetic resonance imaging) ([Figure 1]). Whole body (methoxy-iso butyl-isonitrile) MIBI oncoscintigraphy ([Figure 2] and [Figure 3]) was suggested to re-assess the above bone scan findings. The patient developed sudden onset painless diminution of vision two weeks later. Ophthalmological examination revealed retinal vein thrombosis on right side.
Figure 1. MRI of brain shows features of acute infarct in left frontal lobe (arrow).

Click here to view
Figure 2. Whole body MIBI oncoscintigraphy and 99mTc MDP whole body skeletal scintigraphy showed cold defects in D6 and L4 vertebral bodies confirming scintigraphic diagnosis of bone infarcts.

Click here to view
Figure 3. 99mTC MDP bone SPECT CT (single photon emission computed tomography - computed tomography) images of dorsolumbar vertebrae showing cold defects in D6 and L4 vertebral bodies.

Click here to view


The patient had persistent severe thrombocytopenia to the tune of 15,000 per microliter and elevated serum creatinine of 1.3 mg/dl. The possibilities considered were severe lupus activity with relapse, thrombotic thrombocytopenic purpura (TTP) or severe antiphospholipid antibody syndrome. To rule out TTP, lactate dehydrogenase level was measured which was elevated, but peripheral smear was non contributory ruling out the diagnosis of TTP. She was given three pulses of high-dose steroid despite further deterioration in her platelet counts. Her anticardiolipin antibody titres were positive on two occasions 12 wk apart to the titre of 25-30 MPL units confirming the diagnosis of Hughes syndrome rather than SLE induced bone infarction. The patient was started on intravenous (iv) Heparin (1000 units per hour), titrated based on activated partial thromboplastin time (APTT) values with which she started responding. Platelet counts also stabilized. The patient was doing well at two months follow up.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Article Figures

 Article Access Statistics
    Viewed603    
    Printed8    
    Emailed0    
    PDF Downloaded165    
    Comments [Add]    

Recommend this journal