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Abstract
Discussion Forum (0)
A severe case of neuroleukemiosis in B cell chronic lymphocytic leukemia presenting as mononeuritis multiplex.
Poster No: 1287

Presenter: Alex Vicino
Institution: Lausanne University Hospital

Introduction: A 58 years old woman was diagnosed with B cell chronic lymphocytic leukemia in Mars 2020. She secondarily developed leukemic infiltration in her left breast, granulomatous rosacea (both histologically proven) and bilateral ear chondritis. Blood work disclosed type I cryoglobulinemia and severe hypogammaglobulinemia. In August 2021, she experienced rapidly progressing dysesthesias in her left hand, then to her right hand within a few days. In the two following weeks, she noticed progressive weakness and atrophy in both hands (left predominance), limiting daily activities as bottle-opening. Neurological examination found left predominant amyotrophic paralysis of hand muscles (abductor pollicis brevis M4, dorsal interossei M2), with normal strength elsewhere, normal reflexes and bilateral glove-pattern hypoesthesia.

Methods: Investigations included nerve conduction studies, imaging and nerve biopsy.

Results: Nerve conduction studies were compatible with mononeuritis multiplex with predominantly axonal, non-length dependent and asymmetrical sensorimotor neuropathy. Brachial plexi MRI was normal but brain MRI showed diffuse hypertrophic pachymeningitis ; CSF examination showed pleiocytosis (10 leucocytes, normal <5) with normal protein level. CSF cytology showed 59% of white cells with the same immunophenotype as the known CLL. Radial nerve biopsy showed a diffuse perineural B cell infiltrate (CD20+, CD79a+, CD5+), with the same characteristics found in the previous breast biopsy. Hematologic assessment did not find any evidence of transition to lymphoma. In order to control the severely symptomatic neuroleukemiosis, the patient received steroid and cytoreductive treatment with slight improvement of sensory symptoms (follow-up: 4 weeks).

Conclusions: Mononeuritis multiplex caused by neuroleukemiosis in CLL is an exceptional diagnosis. Because of the positive cryoglubulinemia, we could have considered the diagnosis of peripheral nerve vasculitis and not performed the nerve biopsy. This case supports the interest of nerve biopsy in such rare and atypical cases, as steroids alone in order to treat a suspected vasculitis are not expected to be efficient on the lymphocytic proliferation.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support: N/A

Keywords: Neuroleukemiosis, Mononeuritis multiplex, Chronic lymphocytic leukemia
A severe case of neuroleukemiosis in B cell chronic lymphocytic leukemia presenting as mononeuritis multiplex.
Poster No: 1287

Presenter: Alex Vicino
Institution: Lausanne University Hospital

Introduction: A 58 years old woman was diagnosed with B cell chronic lymphocytic leukemia in Mars 2020. She secondarily developed leukemic infiltration in her left breast, granulomatous rosacea (both histologically proven) and bilateral ear chondritis. Blood work disclosed type I cryoglobulinemia and severe hypogammaglobulinemia. In August 2021, she experienced rapidly progressing dysesthesias in her left hand, then to her right hand within a few days. In the two following weeks, she noticed progressive weakness and atrophy in both hands (left predominance), limiting daily activities as bottle-opening. Neurological examination found left predominant amyotrophic paralysis of hand muscles (abductor pollicis brevis M4, dorsal interossei M2), with normal strength elsewhere, normal reflexes and bilateral glove-pattern hypoesthesia.

Methods: Investigations included nerve conduction studies, imaging and nerve biopsy.

Results: Nerve conduction studies were compatible with mononeuritis multiplex with predominantly axonal, non-length dependent and asymmetrical sensorimotor neuropathy. Brachial plexi MRI was normal but brain MRI showed diffuse hypertrophic pachymeningitis ; CSF examination showed pleiocytosis (10 leucocytes, normal <5) with normal protein level. CSF cytology showed 59% of white cells with the same immunophenotype as the known CLL. Radial nerve biopsy showed a diffuse perineural B cell infiltrate (CD20+, CD79a+, CD5+), with the same characteristics found in the previous breast biopsy. Hematologic assessment did not find any evidence of transition to lymphoma. In order to control the severely symptomatic neuroleukemiosis, the patient received steroid and cytoreductive treatment with slight improvement of sensory symptoms (follow-up: 4 weeks).

Conclusions: Mononeuritis multiplex caused by neuroleukemiosis in CLL is an exceptional diagnosis. Because of the positive cryoglubulinemia, we could have considered the diagnosis of peripheral nerve vasculitis and not performed the nerve biopsy. This case supports the interest of nerve biopsy in such rare and atypical cases, as steroids alone in order to treat a suspected vasculitis are not expected to be efficient on the lymphocytic proliferation.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support: N/A

Keywords: Neuroleukemiosis, Mononeuritis multiplex, Chronic lymphocytic leukemia
A severe case of neuroleukemiosis in B cell chronic lymphocytic leukemia presenting as mononeuritis multiplex.
Dr. Alex Vicino
Dr. Alex Vicino
PNS 2022 Annual Meeting eLibrary. Vicino A. 04/14/2022; 356140; 1287
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Dr. Alex Vicino
Abstract
Discussion Forum (0)
A severe case of neuroleukemiosis in B cell chronic lymphocytic leukemia presenting as mononeuritis multiplex.
Poster No: 1287

Presenter: Alex Vicino
Institution: Lausanne University Hospital

Introduction: A 58 years old woman was diagnosed with B cell chronic lymphocytic leukemia in Mars 2020. She secondarily developed leukemic infiltration in her left breast, granulomatous rosacea (both histologically proven) and bilateral ear chondritis. Blood work disclosed type I cryoglobulinemia and severe hypogammaglobulinemia. In August 2021, she experienced rapidly progressing dysesthesias in her left hand, then to her right hand within a few days. In the two following weeks, she noticed progressive weakness and atrophy in both hands (left predominance), limiting daily activities as bottle-opening. Neurological examination found left predominant amyotrophic paralysis of hand muscles (abductor pollicis brevis M4, dorsal interossei M2), with normal strength elsewhere, normal reflexes and bilateral glove-pattern hypoesthesia.

Methods: Investigations included nerve conduction studies, imaging and nerve biopsy.

Results: Nerve conduction studies were compatible with mononeuritis multiplex with predominantly axonal, non-length dependent and asymmetrical sensorimotor neuropathy. Brachial plexi MRI was normal but brain MRI showed diffuse hypertrophic pachymeningitis ; CSF examination showed pleiocytosis (10 leucocytes, normal <5) with normal protein level. CSF cytology showed 59% of white cells with the same immunophenotype as the known CLL. Radial nerve biopsy showed a diffuse perineural B cell infiltrate (CD20+, CD79a+, CD5+), with the same characteristics found in the previous breast biopsy. Hematologic assessment did not find any evidence of transition to lymphoma. In order to control the severely symptomatic neuroleukemiosis, the patient received steroid and cytoreductive treatment with slight improvement of sensory symptoms (follow-up: 4 weeks).

Conclusions: Mononeuritis multiplex caused by neuroleukemiosis in CLL is an exceptional diagnosis. Because of the positive cryoglubulinemia, we could have considered the diagnosis of peripheral nerve vasculitis and not performed the nerve biopsy. This case supports the interest of nerve biopsy in such rare and atypical cases, as steroids alone in order to treat a suspected vasculitis are not expected to be efficient on the lymphocytic proliferation.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support: N/A

Keywords: Neuroleukemiosis, Mononeuritis multiplex, Chronic lymphocytic leukemia
A severe case of neuroleukemiosis in B cell chronic lymphocytic leukemia presenting as mononeuritis multiplex.
Poster No: 1287

Presenter: Alex Vicino
Institution: Lausanne University Hospital

Introduction: A 58 years old woman was diagnosed with B cell chronic lymphocytic leukemia in Mars 2020. She secondarily developed leukemic infiltration in her left breast, granulomatous rosacea (both histologically proven) and bilateral ear chondritis. Blood work disclosed type I cryoglobulinemia and severe hypogammaglobulinemia. In August 2021, she experienced rapidly progressing dysesthesias in her left hand, then to her right hand within a few days. In the two following weeks, she noticed progressive weakness and atrophy in both hands (left predominance), limiting daily activities as bottle-opening. Neurological examination found left predominant amyotrophic paralysis of hand muscles (abductor pollicis brevis M4, dorsal interossei M2), with normal strength elsewhere, normal reflexes and bilateral glove-pattern hypoesthesia.

Methods: Investigations included nerve conduction studies, imaging and nerve biopsy.

Results: Nerve conduction studies were compatible with mononeuritis multiplex with predominantly axonal, non-length dependent and asymmetrical sensorimotor neuropathy. Brachial plexi MRI was normal but brain MRI showed diffuse hypertrophic pachymeningitis ; CSF examination showed pleiocytosis (10 leucocytes, normal <5) with normal protein level. CSF cytology showed 59% of white cells with the same immunophenotype as the known CLL. Radial nerve biopsy showed a diffuse perineural B cell infiltrate (CD20+, CD79a+, CD5+), with the same characteristics found in the previous breast biopsy. Hematologic assessment did not find any evidence of transition to lymphoma. In order to control the severely symptomatic neuroleukemiosis, the patient received steroid and cytoreductive treatment with slight improvement of sensory symptoms (follow-up: 4 weeks).

Conclusions: Mononeuritis multiplex caused by neuroleukemiosis in CLL is an exceptional diagnosis. Because of the positive cryoglubulinemia, we could have considered the diagnosis of peripheral nerve vasculitis and not performed the nerve biopsy. This case supports the interest of nerve biopsy in such rare and atypical cases, as steroids alone in order to treat a suspected vasculitis are not expected to be efficient on the lymphocytic proliferation.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support: N/A

Keywords: Neuroleukemiosis, Mononeuritis multiplex, Chronic lymphocytic leukemia

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