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Intravascular Lymphoma

Summary

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  • Rare subtype of diffuse large B-cell lymphoma characterised by selective growth of neoplastic cells within blood vessel lumina
  • Presents with nonspecific symptoms, often leading to delayed diagnosis
  • Imaging findings are variable and nonspecific, requiring high clinical suspicion for diagnosis

Pathophysiology

  • Malignant lymphocytes proliferate within the lumen of small blood vessels
  • Exact mechanism of intravascular growth is unclear, but may involve defects in adhesion molecules
  • Leads to occlusion of blood vessels, resulting in ischaemia and organ dysfunction
  • Most commonly affects the central nervous system and skin, but can involve any organ

Demographics

  • Rare disease, with an estimated incidence of less than 1 per million
  • Typically affects older adults, with a median age of 60-70 years
  • No significant gender predilection
  • Slightly higher incidence reported in Asian populations

Diagnosis

  • Often challenging due to nonspecific symptoms and lack of circulating tumour cells
  • Clinical presentation varies depending on organ involvement:
    • CNS: cognitive changes, stroke-like symptoms, seizures
    • Skin: painless erythematous or violaceous patches
    • Systemic: B symptoms (fever, night sweats, weight loss)
  • Laboratory findings may include:
    • Elevated LDH
    • Anaemia
    • Thrombocytopenia
  • Definitive diagnosis requires biopsy of affected tissue with immunohistochemistry

Imaging

  • Findings are nonspecific and variable, depending on organ involvement
  • Central Nervous System:
    • MRI: Multiple infarct-like lesions, often with contrast enhancement
    • Diffusion-weighted imaging may show restricted diffusion
  • Skin:
    • No specific imaging findings; diagnosis typically made by skin biopsy
  • Systemic:
    • PET-CT: May show diffuse FDG uptake in affected organs
    • CT: Nonspecific findings such as hepatosplenomegaly or lymphadenopathy
  • Angiography:
    • May show vascular occlusions or beading of vessels, but rarely performed

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  • 70-year-old patient with progressive right sided weakness.
  • Patchy diffusion restriction and microhaemorrhages in the centrum semiovale without contrast enhancement.

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  • A 50-year-old patient presented with headache and dizziness.
  • MRI showed hazy white matter hyperintensity, puntate diffusion restriction, microhaemorrhages and perivascular enhancement.
  • Intravascular lymphoma was identified following a non-targeted biopsy in the right frontal lobe.

Treatment

  • Systemic chemotherapy is the mainstay of treatment
  • R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisone) is the most commonly used regimen
  • High-dose methotrexate may be added for CNS involvement
  • Autologous stem cell transplantation may be considered in eligible patients
  • Prognosis is generally poor, with a median survival of 12-18 months without treatment
  • Early diagnosis and prompt initiation of therapy can improve outcomes

Differential diagnosis

Differential Diagnosis Differentiating Feature
Multiple sclerosis Typical demyelinating lesions on MRI; intravascular lymphoma shows multifocal infarcts
Embolic stroke Often has an identifiable embolic source; intravascular lymphoma has no clear source
Primary CNS angiitis Angiography shows beading of vessels; biopsy shows inflammation of vessel walls
Susac syndrome Retinal artery occlusions and hearing loss; specific corpus callosum lesions on MRI