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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphoma, Intravascular | 11A0 Case 11 History
Case 11 History ---- The patient was a 74 year old man with a history of insulin-dependent diabetes mellitus, prior left cerebral vascular accident (fully resolved), hypothyroidism, and ethanol and tobacco abuse who was transferred from an outside hospital on 11/30 with depressed mental status and hypotension. He had been admitted to an OSH on 10/29 with the diagnosis of a "viral syndrome". He was treated for dehydration and discharged in his usual state of health, but he was then readmitted on 11/14 with leg pain. A right kidney stone passed spontaneously on 11/22; thereafter, his daughter noted progressively declining mental status. The patient had been receiving Demerol for kidney stone pain, which was stopped. A head CT revealed bilateral basal ganglia infarcts unchanged from a previous scan on 10/31. Notable lab data included a CBC which showed elevated reticulocytes, anemia, and thrombocytopenia. The patient had an episode of hypotension on 11/28 which responded to fluid administration. A second episode of hypotension on 11/30 was accompanied by respiratory failure and the patient was transferred to BJH for further care. On admission the patient was agitated and in acute respiratory distress. His blood pressure was 95/70, pulse 100, temp 35.2, and respiration rate 20-30. He had nuchal rigidity, diffuse wheezes, and a tender epigastrium with hypoactive bowel sounds. He was lethargic and intermittently followed simple commands. Lab data included PaO2 of 65 on room air, platelets of 44K, WBC of 6.8, H/H 9.8/29.6, PT 15/PTT 28.5, Cr 3.0, BUN 94, K 5.2. CSF analysis was not suggestive of meningitis, but the patient was started on broad spectrum antibiotics for presumed sepsis. Shortly after admission the patient required intubation and pressors. General surgery felt that primary bowel ischemia was unlikely. A chest x-ray showed pulmonary edema. The patient became febrile; urine cultures grew Staph aureus and sputum cultures grew gram negative bacilli. The patient developed DIC with worsening thrombocytopenia. He developed ascites and paracentesis was performed. He continued to be hypotensive despite aggressive use of pressors. An echocardiogram ruled-out cardiac effusion/tamponade. On 12/4 he progressed to complete hemodynamic collapse and proceeded to cardiac arrest. ---- At autopsy the weight of the unfixed brain was 1350g. Coronal sections of the cerebral hemispheres revealed multiple cortical and subcortical lesions. Virtually all of the tissue sections show scattered blood vessels filled with malignant lymphoid cells. An immunohistochemical stain for L26 (a B-cell marker) stains virtually all of the malignant cells while an immunohistochemical stain for UCHLl (a T-cell marker) is negative in these cells. This demonstrates that the malignant cells are of B-cell origin. There is no evidence of intraparenchymal infiltration of these neoplastic cells as they appear to be confined to the vascular spaces. Similarly, the general autopsy report documents that there is no identifiable tissue mass lesion and thus this neoplasm is best characterized as an intravascular lymphoma of B-cell lineage. There is an acute/subacute hemorrhagic infarct of the right occipital parietal white matter and multifocal remote cystic infarcts that are completely resolved and are accompanied by hyaline arteriolosclerosis.
