A 61-year-old man with hypertension under treatment.
On day 5, she presented with fluctuating consciousness and decreased strength in the right upper limb.
The lower limbs were abolished.
Complete blood count showed normocytic normochromic anaemia (haemoglobin: 9.8 g/dL, haematocrit - HSu: 29.9%, HCM: 32 pg, MCV: 91 fL), white blood cell count
Lumbar puncture (LP) was performed, highlighting total proteins of 59.4 mg/dL, without pleocytosis.
The abdominal echotomography showed a heterogeneous liver structure, without focal lesions, enlarged spleen and bilateral hydronephrosis.
Upper endoscopy showed esophageal varices grade I and hypertensive gastropathy.
Chest CT showed no pathological findings.
After 11 days, the patient remained confused, with brachypsychic, dysthymic and bilateral paresis of the lower limbs, aggregating in the following days tonic and presenting convulsion.
CT and magnetic resonance imaging (MRI) of the brain showed subacute infarcts of both cerebellar hemispheres in territories cerebellar tumeal mass, inferior tense and hemorrhagic transformation with moderate effect.
In addition, multiple subacute ischemic focal lesions were demonstrated in territories of borderline circulation of both cerebral hemispheres and partially cavitated left tempora-parietal subacute infarction.
In the absence of signs of meningeal irritation, a new LP was performed, highlighting total proteins of 98.7 mg/dL, without pleocytosis, with smear microscopy and culture for M. tuberculosis negative.
Tests for HIV, VDRL and HTLV were also negative.
The patient remained conscious, with fever, tachypnea and cough fluctuating pulmonary examination compatible with pneumonia, and antibiotic treatment was initiated.
Finally, after 20 days the patient died, raising the clinical suspicion of congophilic cerebral angiopathy.
Postmortem examination revealed infarctions in the left temporoparietal and cerebellar bilateral level.
Microscopic examination revealed the presence of small and medium blood vessels in the lumen of atypical medium-large lymphocytes that partially or totally filled their lumen, particularly in relation to infarcted areas.
In the pancreas, liver, kidney, adrenal glands, myocardium, lung and thyroid gland intravascular tumor involvement was also observed.
Hemophagocytosis was observed in the bone marrow, but tumor necrosis was not demonstrated.
Immunohistochemical study with CD45, CD3, CD20 and CD79a showed positivity in intravascular tumor lymphocytes for CD45, CD20 and CD79a, findings consistent with intravascular large B-cell lymphoma.
