PARANEOPLASTIC CEREBELLAR DEGENERATION ASSOCIATED WITH HODGKIN LYMPHOMA : A FRENCH RETROSPECTIVE COHORT
Author(s): ,
Kentin Queru
Affiliations:
Department of Hematology,CHU Dupuytren Limoges,Limoges,France
,
Bastien Joubert
Affiliations:
French Reference Center on Paraneoplastic Neurological Syndrome,Hospices Civils de Lyon,Lyon,France
,
Géraldine Picard
Affiliations:
French Reference Center on Paraneoplastic Neurological Syndrome,Hospices Civils de Lyon,Lyon,France
,
Véronique Rogemont
Affiliations:
French Reference Center on Paraneoplastic Neurological Syndrome,Hospices Civils de Lyon,Lyon,France
,
Mohammed Touati
Affiliations:
Department of Hematology,CHU Dupuytren Limoges,Limoges,France
,
Arnaud Jaccard
Affiliations:
Department of Hematology,CHU Dupuytren Limoges,Limoges,France
,
Laurent Magy
Affiliations:
Department of Neurology,CHU Dupuytren Limoges,Limoges,France
,
Julie Abraham
Affiliations:
Department of Hematology,CHU Dupuytren Limoges,Limoges,France
Jerome Honnorat
Affiliations:
French Reference Center on Paraneoplastic Neurological Syndrome,Hospices Civils de Lyon,Lyon,France
(Abstract release date: 05/14/20) EHA Library. Queru K. 06/12/20; 293629; EP1140
Mr. Kentin Queru
Mr. Kentin Queru
Contributions
Abstract

Abstract: EP1140

Type: e-Poster

Background
Paraneoplastic cerebellar degeneration (PCD) is reported as a rare but severe autoimmune complication in Hodgkin lymphoma (HL). The majority of patients have autoantibodies against neural antigens in their serum and/or CSF, notably anti-Tr/DNER antibody is a strong biological marker of HL with PCD.

Aims
Few cases describe clinical and therapeutic aspects of this entity. We report the first retrospective cohort of PCD secondary to HL describing hematological aspect.

Methods
We reviewed the clinicobiologic characterics of 20 patients presented PCD preceding HL and whose serum or CSF (cerebrospinal fluid) had been sent to the French reference center on paraneoplastic neurological syndromes (from 2001 to 2019) to detect anti-neuronal antibodies from 15 centers.

Results
Patients were mainly males (n=17), median age of 45 years [24-72], with a median follow up of 3,75 years [1-15]. No major co-morbidities were reported except for 1 patient who had HL in remission since 10 years. In all cases except 1, neurological signs preceded HL diagnosis. The PCD presentation was, firstly cephalea and dizzness that rapidly progress to severe symmetrical ataxia, dysarthria, diplopia and downt-beat nystagmus. The whole PCD settled down in 2-3 weeks and was isolated in all cases. Rankin score was rapidly worsening with score ≥3 for 17 patients at HL diagnosis. Majority of brain MRI was normal at diagnostic, 2 showed FLAIR hyperintensities cerebellar lesions and 3 global cerebellar atrophia. CSF presented pleiocytosis of lymphocytes. 16 patients had anti-Tr/DNER antibody positive in CSF, 1 neuropile and 3 without antibody found. A 5-week delay was necessary to deliver the results of antibody. Median delay between first neurological symptoms and diagnosis of HL was 14 weeks [1-35]. Clinically, at least 8 patients had palpable lymphadenopathy. After PETscan evaluation, 14 have early stage HL, 4 advanced and 3 data-free. Regarding the histological subtype, all are classical type: 8 nodular sclerosis, 4 mixed cellulary, 1 lymphocyte rich and 7 unspecified. Except 3 early deaths by sepsis (direct chemotherapy complication), all patients were in long-remission after first-line treatment and 1 patient relapsed 5 years after second remission. Regarding HL treatment, 12 patients received ABVD with or without radiotherapy, 3 BEACOPP, 1 CHOP. Regarding PCD treatment, 13 patients received monthly cycles of intravenous immunoglobulin G (IVIg) (0.4mg/kg/d for 5 days). Median was 4 cycles [1-18]. Six patients received Rituximab (1g/m2 D0-D15), all of them received IVIg in association. Median was 2 cycles [1-6]. One patient received belatedly Solumedrol 1g/j during 5 days at HL diagnosis without real neurological amelioration. Finally, for 10 patients PCD partially regressed whereas 7 patients had unimproved PCD. The HL stage doesn’t appear to correlate with the severity of PCD and the neurological recovery of the patient. The longer the interval between diagnosis and therapy is, the more severe and irreversible the lesions are. Combination of chemotherapy and IVIG would allow a halt to the worsening of PCD and a significant early improvement. IVIG early, less than 6 weeks after PCD onset, with chemotherapy seems improve quickly PCD.

Conclusion
PCD complication of HL is rare but probably underdiagnosed. It doesn’t seem to impact the hematological prognosis of HL whereas early death linked to chemotherapy complication could be favored by the neurological state. Functional neurological prognosis depends on the delay of diagnosis, early delivery of IVIg and specific chemotherapy is important for outcomes.

Session topic: 17. Hodgkin lymphoma - Clinical

Keyword(s): Complications, Hodgkin's lymphoma

Abstract: EP1140

Type: e-Poster

Background
Paraneoplastic cerebellar degeneration (PCD) is reported as a rare but severe autoimmune complication in Hodgkin lymphoma (HL). The majority of patients have autoantibodies against neural antigens in their serum and/or CSF, notably anti-Tr/DNER antibody is a strong biological marker of HL with PCD.

Aims
Few cases describe clinical and therapeutic aspects of this entity. We report the first retrospective cohort of PCD secondary to HL describing hematological aspect.

Methods
We reviewed the clinicobiologic characterics of 20 patients presented PCD preceding HL and whose serum or CSF (cerebrospinal fluid) had been sent to the French reference center on paraneoplastic neurological syndromes (from 2001 to 2019) to detect anti-neuronal antibodies from 15 centers.

Results
Patients were mainly males (n=17), median age of 45 years [24-72], with a median follow up of 3,75 years [1-15]. No major co-morbidities were reported except for 1 patient who had HL in remission since 10 years. In all cases except 1, neurological signs preceded HL diagnosis. The PCD presentation was, firstly cephalea and dizzness that rapidly progress to severe symmetrical ataxia, dysarthria, diplopia and downt-beat nystagmus. The whole PCD settled down in 2-3 weeks and was isolated in all cases. Rankin score was rapidly worsening with score ≥3 for 17 patients at HL diagnosis. Majority of brain MRI was normal at diagnostic, 2 showed FLAIR hyperintensities cerebellar lesions and 3 global cerebellar atrophia. CSF presented pleiocytosis of lymphocytes. 16 patients had anti-Tr/DNER antibody positive in CSF, 1 neuropile and 3 without antibody found. A 5-week delay was necessary to deliver the results of antibody. Median delay between first neurological symptoms and diagnosis of HL was 14 weeks [1-35]. Clinically, at least 8 patients had palpable lymphadenopathy. After PETscan evaluation, 14 have early stage HL, 4 advanced and 3 data-free. Regarding the histological subtype, all are classical type: 8 nodular sclerosis, 4 mixed cellulary, 1 lymphocyte rich and 7 unspecified. Except 3 early deaths by sepsis (direct chemotherapy complication), all patients were in long-remission after first-line treatment and 1 patient relapsed 5 years after second remission. Regarding HL treatment, 12 patients received ABVD with or without radiotherapy, 3 BEACOPP, 1 CHOP. Regarding PCD treatment, 13 patients received monthly cycles of intravenous immunoglobulin G (IVIg) (0.4mg/kg/d for 5 days). Median was 4 cycles [1-18]. Six patients received Rituximab (1g/m2 D0-D15), all of them received IVIg in association. Median was 2 cycles [1-6]. One patient received belatedly Solumedrol 1g/j during 5 days at HL diagnosis without real neurological amelioration. Finally, for 10 patients PCD partially regressed whereas 7 patients had unimproved PCD. The HL stage doesn’t appear to correlate with the severity of PCD and the neurological recovery of the patient. The longer the interval between diagnosis and therapy is, the more severe and irreversible the lesions are. Combination of chemotherapy and IVIG would allow a halt to the worsening of PCD and a significant early improvement. IVIG early, less than 6 weeks after PCD onset, with chemotherapy seems improve quickly PCD.

Conclusion
PCD complication of HL is rare but probably underdiagnosed. It doesn’t seem to impact the hematological prognosis of HL whereas early death linked to chemotherapy complication could be favored by the neurological state. Functional neurological prognosis depends on the delay of diagnosis, early delivery of IVIg and specific chemotherapy is important for outcomes.

Session topic: 17. Hodgkin lymphoma - Clinical

Keyword(s): Complications, Hodgkin's lymphoma

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