1Sara Ali Alshamali, 1Jihad Said Salim Inshasi
1Rashid Hospital, Dubai Health Authority, Dubai, United Arab Emirates
CNS involvement in CLL is rare and it usually occurs in late-stage CLL disease. There is usual delay in the diagnosis due to its variable manifestations, challenging diagnosis process and possible misdiagnosis with a mimicker condition. I am sharing our relative successful experience with this challenging case that had satisfied outcome after going through comprehensive investigations and treatment journey treating his symptoms until arriving the final diagnosis and getting the best treatment option.
Material(s) and Method(s):
A 42 years old male, with recent COVID-19 infection, presented with multiple progressive neurologic symptoms over one month; started as numbness around the mouth, reduced facial sensation and a feeling of band like sensation below the costal margins. On exam, he had left abduction restriction, diplopia on left gaze and upbeat nystagmus, reduced facial sensation and hyperesthesia. The reflexes were 1+ in the upper limbs, 3+ in the lower limbs, up going planters, tingling from the feet up to T6 level and postural tremor bilaterally.
His CSF showed high protein level. MRI brain/ spine revealed left frontal juxtacortical white matter and bilateral middle cerebral peduncles lesions with post-contrast enhancement and long segment spinal cord demyelinating plaques.
He was initially treated as a case of Acute disseminated encephalomyelitis (ADEM) post viral infection in a background of CLL. The delayed diagnosis was due to temporal relation of neurological manifestation to viral infection, similar MRI lesions to ADEM and multiple negative CSF results of cytology and flow cytometry.
He had persistent disabling symptoms and enhancing lesions in MRI despite being treated with IVMP, IVIG and PLEX.
He was managed for ADEM based on responsiveness to the recommended therapy step by step. Firstly, he received a high-dose corticosteroids, secondly IV immunoglobulin but he was still progressing and considered as steroid-unresponsive ADEM. lastly, plasma exchange was done when he exhibited progressive symptoms with fair improvement.
Interestingly, the patient showed significant improvement in the clinical and radiological parameters after starting him with a new anti-leukemia medication (Acalabrutinib) for his concurrent active condition.
He run out of his medication for around 1 week and he experienced recurrent of the neurological manifestation and the previous lesions in the images. A repeated flow cytometry for the third time came positive for CLL cells and the final diagnosis of CNS involvement by CLL was established. The diagnosis was made after the exclusion of other etiologies.
The patient received Ibrutinib at a standard dose and as a monotherapy. It is an efficient chemotherapy that crosses the blood brain barrier and has showed a favorable clinical, biological and radiological outcome. The patient is back to his work and his daily activities have improved.
In case of inconclusive work up, CSF analysis should be repeated testing for cytology and flow cytometry\immunophenotypes as the false negative results are common.
Our patient had an active CLL proved in his investigations, and the fact that the patient responded very well to the new chemotherapy should alert the diagnosis of CNS involvement by CLL and directs towards repeating investigations and introducing aggressive treatment strategy to target both hematological and neurological complications of the condition.