Contributions
Abstract: PB1561
Type: Publication Only
Session title: Hodgkin lymphoma - Clinical
Background
Charcot-Marie-Tooth (CMT) disease represents a heterogeneous group of disorders of peripheral nerve, that is characterized by progressive weakness and atrophy of distal muscles, loss of tendon reflexes, mild sensory impairment and foot deformity.
Two major types of CMT can be differentiated: type I (IA chromosome 17p11.2 gen PMP22, IB chromosome 1q22-23 gen P0, IC unknown gen) more frequently (60%) and type II. Both types are inherited in an autosomal dominant pattern and generally show a benign and chronic clinical course.
Aims
To show severe and life-threatening neuropathic events in patients with hereditary neuropathies receiving Brentuximab-Vedotin (BV).
Methods
We report an unusual case of acute deterioration of CMT disease IA following three doses of BV.
Results
A 27-year-old male was diagnosed with Hodgkin’s lymphoma in June 2020. He presented cervical lymphadenopathies. Definite histological diagnosis was made after lymph node removal. Further diagnostic studies revealed splenic and bone infiltration and he was staged IVA (Ann Arbor Classification). BV+AVD-chemotherapy (Adriamycin, Bleomycin, Vinblastine) was the chosen treatment; 120 mg was the BV dose administered in each cycle (1,2 mg/kg). At that point, the neurological status and clinical examination results showed no alterations. No family or personal history was notified.
He received two doses of BV without problems. The third dose was given on 11 September. Three days later, the patient started with neurological symptoms such as muscular weakness, both in the upper and lower limbs (power grade III), fingertips’ paresthesia, bilateral sensory impairment of feet and lower legs. Symptoms worsened significantly resulting in dependency of a wheelchair. Finally, he was assessed by a neurologist and diagnostic studies were performed. He continued with the treatment for his lymphoma without BV and with a provisional diagnosis of severe BV-induced neuropathy. At this moment, his father revealed that he had a Charcot-Marie-Tooth disease.
Electrophysiological studies showed sensory-motor polyneuropathy with predominantly demyelinating. We searched his medical history from children’s hospital and it was observed that he presented 17p11.2 duplication typical for CMT disease IA. While continuing chemotherapy without the use of BV, the patient's neurological symptoms slowly recovered with rehabilitation for four months.
Conclusion
Peripheral neuropathy induced by BV is a characteristic effect of cumulative exposure to this drug and it is reversible in most cases. As shown in our patient the coincidence of BV use and underlying CMT IA can lead to severe neurological impairment. If these symptoms are unusual or do not recover before the next dose of BV, diagnostic work-up for underlying hereditary neuropathy including nerve conduction velocity studies and sometimes molecular genetic analysis should be performed. No further use of BV or at least adequate dose reduction have also to be considered. However, we have not found cases like ours on bibliography. Only a few cases have been reported with the use of vincristine.
We conclude that a detailed family history and careful clinical examination should be obtained before exposing patients to any kind of neurotoxic agents. Otherwise, consequences can be deleterious for the quality of patient’s life.
Keyword(s): Hodgkin's lymphoma, Toxicity
Abstract: PB1561
Type: Publication Only
Session title: Hodgkin lymphoma - Clinical
Background
Charcot-Marie-Tooth (CMT) disease represents a heterogeneous group of disorders of peripheral nerve, that is characterized by progressive weakness and atrophy of distal muscles, loss of tendon reflexes, mild sensory impairment and foot deformity.
Two major types of CMT can be differentiated: type I (IA chromosome 17p11.2 gen PMP22, IB chromosome 1q22-23 gen P0, IC unknown gen) more frequently (60%) and type II. Both types are inherited in an autosomal dominant pattern and generally show a benign and chronic clinical course.
Aims
To show severe and life-threatening neuropathic events in patients with hereditary neuropathies receiving Brentuximab-Vedotin (BV).
Methods
We report an unusual case of acute deterioration of CMT disease IA following three doses of BV.
Results
A 27-year-old male was diagnosed with Hodgkin’s lymphoma in June 2020. He presented cervical lymphadenopathies. Definite histological diagnosis was made after lymph node removal. Further diagnostic studies revealed splenic and bone infiltration and he was staged IVA (Ann Arbor Classification). BV+AVD-chemotherapy (Adriamycin, Bleomycin, Vinblastine) was the chosen treatment; 120 mg was the BV dose administered in each cycle (1,2 mg/kg). At that point, the neurological status and clinical examination results showed no alterations. No family or personal history was notified.
He received two doses of BV without problems. The third dose was given on 11 September. Three days later, the patient started with neurological symptoms such as muscular weakness, both in the upper and lower limbs (power grade III), fingertips’ paresthesia, bilateral sensory impairment of feet and lower legs. Symptoms worsened significantly resulting in dependency of a wheelchair. Finally, he was assessed by a neurologist and diagnostic studies were performed. He continued with the treatment for his lymphoma without BV and with a provisional diagnosis of severe BV-induced neuropathy. At this moment, his father revealed that he had a Charcot-Marie-Tooth disease.
Electrophysiological studies showed sensory-motor polyneuropathy with predominantly demyelinating. We searched his medical history from children’s hospital and it was observed that he presented 17p11.2 duplication typical for CMT disease IA. While continuing chemotherapy without the use of BV, the patient's neurological symptoms slowly recovered with rehabilitation for four months.
Conclusion
Peripheral neuropathy induced by BV is a characteristic effect of cumulative exposure to this drug and it is reversible in most cases. As shown in our patient the coincidence of BV use and underlying CMT IA can lead to severe neurological impairment. If these symptoms are unusual or do not recover before the next dose of BV, diagnostic work-up for underlying hereditary neuropathy including nerve conduction velocity studies and sometimes molecular genetic analysis should be performed. No further use of BV or at least adequate dose reduction have also to be considered. However, we have not found cases like ours on bibliography. Only a few cases have been reported with the use of vincristine.
We conclude that a detailed family history and careful clinical examination should be obtained before exposing patients to any kind of neurotoxic agents. Otherwise, consequences can be deleterious for the quality of patient’s life.
Keyword(s): Hodgkin's lymphoma, Toxicity