Abstract 4784

Background

Patients with classical Hodgkin Lymphoma (cHL) have a relatively high risk of venous thrombo-embolism (VTE); reported incidence 4.6–7% in adults and up to 11.5% in children and adolescents. Most VTE episodes are peripheral or related to central venous catheters, with very limited data on central or life-threatening thromboses in adolescents. There is only 1 reported case series on cerebral venous thrombosis (CVT) in adolescents. We report 4 cases of CVT from our centre, all treated with chemo-radiotherapy. Chemotherapy comprised OEPA (vincristine, prednisolone, doxorubicin, etoposide) and COPP/COPDAC (cyclophosphamide, vincristine, prednisolone, procarbazine/dacarbazine respectively).

Table 1:

Clinical details

PatientAge at diagnosis of cHLStageChemotherapyPeripheral VTEOn LMWH at time of CVT
1 12 IIAE 2 OEPA No No 
2 17 IIIBE 2 OEPA + 4 COPP Yes PICC associated during OEPA #2 No 
3 18 IVBE 2 OEPA + 4 COPDAC No No 
4 23 IIB 2 OEPA + 2 COPP Yes PICC associated during OEPA #1 No 
PatientAge at diagnosis of cHLStageChemotherapyPeripheral VTEOn LMWH at time of CVT
1 12 IIAE 2 OEPA No No 
2 17 IIIBE 2 OEPA + 4 COPP Yes PICC associated during OEPA #2 No 
3 18 IVBE 2 OEPA + 4 COPDAC No No 
4 23 IIB 2 OEPA + 2 COPP Yes PICC associated during OEPA #1 No 
View Large
Results

All patients received involved field radiotherapy (IFRT) 19.8 – 30Gy on completing chemotherapy. All were female, aged 12–23. All received norethisterone contraception. All had indwelling central venous catheters (PICC). Patient 4 alone had a raised body mass index. All were exposed to steroids; Patient 4 completed steroid therapy several weeks before developing CVT symptoms. Patients 2 and 4 received treatment dose low molecular weight heparin (LMWH) for 6 weeks after diagnosis of PICC-associated thrombosis, and were not on anticoagulation or thromboprophylaxis when CVT was diagnosed. Regarding other risk factors, 3/4 had no documented prothrombotic tendency. Patient 4 was found to have a moderately positive IgM anti beta 2 glycoprotein antibody present 12 weeks apart, consistent with antiphospholipid syndrome.

Table 2:

Clinical Details

PatientTreatment at time of CVTClinical PresentationImaging reportAcute Treatment
1 OEPA #2 Dizzy spell, loss of vision right eye, right hyperacusis, right sided headache, hoarse voice, left 6th cranial nerve palsy, raised intracranial pressure Right transverse sinus and superior sagittal sinus thrombosis LMWH, acetazolamide, therapeutic lumbar punctures 
2 COPP #2 Severe headache, bilateral papilloedema, tonic clonic seizures, raised intracranial pressure Superior sagittal sinus thrombus with extension into left transverse and left sigmoid sinus LMWH, acetazolamide + bicarbonate, therapeutic lumbar punctures, phenytoin 
3 OEPA #1 Malaise, headache, weakness left upper and lower limbs, tonic clonic seizures Superior sagittal sinus and right transverse sinus thrombosis extending into cortical veins with right parietal intracranial haematoma LMWH, thrombectomy with tissue plasminogen activator, phenytoin 
4 Post Radiotherapy Headache, photophobia, blurred vision, drowsiness, recurrent collapse, ataxia Superior sagittal sinus thrombosis, extension into transverse venous sinuses, with involvement of right sigmoid sinus and internal jugular vein LMWH with warfarin loading 
PatientTreatment at time of CVTClinical PresentationImaging reportAcute Treatment
1 OEPA #2 Dizzy spell, loss of vision right eye, right hyperacusis, right sided headache, hoarse voice, left 6th cranial nerve palsy, raised intracranial pressure Right transverse sinus and superior sagittal sinus thrombosis LMWH, acetazolamide, therapeutic lumbar punctures 
2 COPP #2 Severe headache, bilateral papilloedema, tonic clonic seizures, raised intracranial pressure Superior sagittal sinus thrombus with extension into left transverse and left sigmoid sinus LMWH, acetazolamide + bicarbonate, therapeutic lumbar punctures, phenytoin 
3 OEPA #1 Malaise, headache, weakness left upper and lower limbs, tonic clonic seizures Superior sagittal sinus and right transverse sinus thrombosis extending into cortical veins with right parietal intracranial haematoma LMWH, thrombectomy with tissue plasminogen activator, phenytoin 
4 Post Radiotherapy Headache, photophobia, blurred vision, drowsiness, recurrent collapse, ataxia Superior sagittal sinus thrombosis, extension into transverse venous sinuses, with involvement of right sigmoid sinus and internal jugular vein LMWH with warfarin loading 
View Large

All patients were therapeutically anticoagulated for 6 months to 1 year. LMWH of choice at our centre was dalteparin. Patient 1 was switched to warfarin upon completion of chemo-radiotherapy, and Patient 4 was commenced on warfarin with dalteparin cover at diagnosis of CVT as she had completed treatment.

Patients 1 and 2 had raised intracranial pressure on lumbar puncture, and required therapeutic lumbar punctures and acetazolamide.

Patients 2 and 3 both required anticonvulsant therapy for 1 year. Patient 2 was initially treated with phenytoin, and switched to carbamazepine. Patient 3 was also initially managed with phenytoin, and switched to levetiracetam. Neither patient had any subsequent seizures.

All 4 patients have recovered completely from CVT with no residual neurological deficits or further thromboses.

Conclusion

CVT is a rare and potentially life threatening complication in adolescents and young adults with cHL with paucity of data. The risk factors are unclear however all patients in our series were female, received steroids and were on norethisterone. Only 1 patient had a prothrombotic tendency detected on thrombophila screening. CVT is treatable, and complete resolution of signs and symptoms can be expected. More studies are required to elucidate risk factors which may help develop thromboprophylaxis guidance in this group of patients.

Disclosures:

No relevant conflicts of interest to declare.

Topics:
adolescent, cerebral venous thrombosis, hodgkin's disease, young adult, low-molecular-weight heparin, cyclophosphamide/prednisone/procarbazine/vincristine, diagnostic spinal puncture, headache, peripherally inserted central catheter, phenytoin

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2012 by The American Society of Hematology
2012
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