Abstract:Guillain-Barré syndrome (GBS) is a rare condition caused by autoimmune damage of peripheral nerves. We describe a case where a man in his 80s presented with subacute, progressive fatigue and weakness. He had received an outpatient work-up for possible haematological malignancy, but eventually presented to the emergency department for worsening weakness. A physical exam and cerebrospinal fluid analysis suggested a diagnosis of GBS. Subsequently, a pathological diagnosis of angioimmunoblastic T-cell lymphoma was… Show more
“…Given its vague symptomatology, IVLBCL often elicits a wide array of differential diagnoses, including hyponatremia, stroke, adrenal insufficiency, thyroid disease, polymyositis, vasculitis, and other connective tissue diseases. Other malignancies may also present similarly to our patient's presentation, as demonstrated by a case of Guillain-Barre Syndrome caused by angioimmunoblastic T-cell lymphoma shown in a recent report [5]. As such, the diagnosis of this disease is a lengthy and arduous process.…”
Section: Discussionsupporting
confidence: 78%
“…Surgical excision is preferred over punch biopsy as the latter often does not incorporate enough hypodermis tissue to reveal the intravascular tumor cells. Classical pathological findings of IVLBCL involve clumps of large abnormal lymphocytes coalescing in the lumen of small to medium-sized vessels [ 5 ]. Immunohistochemical staining shows strong pan-B-cell markers including CD5 (20–50%), BCL-2 (>90%), c-MYC (˜70%), MUM-1/IRF-4 (˜75%), BCL-6 (25–60%), and CD10 (˜10%) [ 1 ].…”
We are reporting the first documented case of intravascular large B-cell lymphoma (IVLBCL) manifesting in the endomysial and perimysial capillaries with its associated diagnostic dilemma. Our patient presented with progressive paraplegia. Initial laboratories were remarkable for hyponatremia, hypochloremia, lactic acidosis, elevated C-reactive protein, and lactate dehydrogenase. The bone marrow biopsy was unrevealing. However, a subsequent muscle biopsy confirmed the diagnosis of IVLBCL. As hyponatremia, endocrinopathies, connective tissue disease, rheumatological disorders, and occult cancer could all present similarly, our patient is a unique diagnostic dilemma. Randomized skin biopsy remains the best way to diagnose this disease, and rituximab-based chemotherapy with high-dose intrathecal methotrexate has proven to be a safe and effective regimen. With this initial evidence of IVLBCL involving the endomysial and perimysial capillary, we believe that muscle biopsy could be of value in diagnosing IVLBCL patients with neuromuscular symptoms.
“…Given its vague symptomatology, IVLBCL often elicits a wide array of differential diagnoses, including hyponatremia, stroke, adrenal insufficiency, thyroid disease, polymyositis, vasculitis, and other connective tissue diseases. Other malignancies may also present similarly to our patient's presentation, as demonstrated by a case of Guillain-Barre Syndrome caused by angioimmunoblastic T-cell lymphoma shown in a recent report [5]. As such, the diagnosis of this disease is a lengthy and arduous process.…”
Section: Discussionsupporting
confidence: 78%
“…Surgical excision is preferred over punch biopsy as the latter often does not incorporate enough hypodermis tissue to reveal the intravascular tumor cells. Classical pathological findings of IVLBCL involve clumps of large abnormal lymphocytes coalescing in the lumen of small to medium-sized vessels [ 5 ]. Immunohistochemical staining shows strong pan-B-cell markers including CD5 (20–50%), BCL-2 (>90%), c-MYC (˜70%), MUM-1/IRF-4 (˜75%), BCL-6 (25–60%), and CD10 (˜10%) [ 1 ].…”
We are reporting the first documented case of intravascular large B-cell lymphoma (IVLBCL) manifesting in the endomysial and perimysial capillaries with its associated diagnostic dilemma. Our patient presented with progressive paraplegia. Initial laboratories were remarkable for hyponatremia, hypochloremia, lactic acidosis, elevated C-reactive protein, and lactate dehydrogenase. The bone marrow biopsy was unrevealing. However, a subsequent muscle biopsy confirmed the diagnosis of IVLBCL. As hyponatremia, endocrinopathies, connective tissue disease, rheumatological disorders, and occult cancer could all present similarly, our patient is a unique diagnostic dilemma. Randomized skin biopsy remains the best way to diagnose this disease, and rituximab-based chemotherapy with high-dose intrathecal methotrexate has proven to be a safe and effective regimen. With this initial evidence of IVLBCL involving the endomysial and perimysial capillary, we believe that muscle biopsy could be of value in diagnosing IVLBCL patients with neuromuscular symptoms.
“…3 : The tumor follicular T-helper cells activate B cells, which turn into plasma cells and produce large amounts of antibodies. Furthermore, in several case reports AIDP has been linked to AITL [ 8 , 9 ]. Fig.…”
Guillain–Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory infection. This narrative overview aims to summarise and discuss current knowledge and previous evidence regarding triggers and pathophysiology of GBS. A systematic search of the literature was carried out using suitable search terms. The most common subtypes of GBS are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The most common triggers of GBS, in three quarters of cases, are previous infections. The most common infectious agents that cause GBS include Campylobacter jejuni (C. jejuni), Mycoplasma pneumoniae, and cytomegalovirus. C. jejuni is responsible for about a third of GBS cases. GBS due to C. jejuni is usually more severe than that due to other causes. Clinical presentation of GBS is highly dependent on the structure of pathogenic lipo-oligosaccharides (LOS) that trigger the innate immune system via Toll-like-receptor (TLR)-4 signalling. AIDP is due to demyelination, whereas in AMAN, structures of the axolemma are affected in the nodal or inter-nodal space. In conclusion, GBS is a neuro-immunological disorder caused by autoantibodies against components of the myelin sheath or axolemma. Molecular mimicry between surface structures of pathogens and components of myelin or the axon is one scenario that may explain the pathophysiology of GBS.
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