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Hughes syndrome with cerebral, skeletal infarction & retinal vein thrombosis.

Sundaram PS, Padma S - Indian J. Med. Res. (2015)

View Article: PubMed Central - PubMed

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

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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... A Technetium 99m (Tc 99m)-methylene diphosphonate (MDP) bone scan was advised... 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.

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MRI of brain shows features of acute infarct in left frontal lobe (arrow).
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Figure 1: MRI of brain shows features of acute infarct in left frontal lobe (arrow).

Mentions: 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) (Fig. 1). Whole body (methoxy-iso butyl-isonitrile) MIBI oncoscintigraphy (Figs 2 and 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.


Hughes syndrome with cerebral, skeletal infarction & retinal vein thrombosis.

Sundaram PS, Padma S - Indian J. Med. Res. (2015)

MRI of brain shows features of acute infarct in left frontal lobe (arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4418167&req=5

Figure 1: MRI of brain shows features of acute infarct in left frontal lobe (arrow).
Mentions: 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) (Fig. 1). Whole body (methoxy-iso butyl-isonitrile) MIBI oncoscintigraphy (Figs 2 and 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.

View Article: PubMed Central - PubMed

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

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

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... A Technetium 99m (Tc 99m)-methylene diphosphonate (MDP) bone scan was advised... 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.

No MeSH data available.


Related in: MedlinePlus