Intravascular lymphomatosis: a case presenting with encephalomyelitis and reactive haemophagocytic syndrome diagnosed by renal biopsy

• Published in: Histopathology Vol. 31; no. 6; pp. 552 - 554
• Main Authors: CHENG, F.Y., TSUI, W.M.S., YEUNG, W.T.C., IP, L.S., NG, C.S.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science Ltd 01.12.1997; Blackwell
• Subjects: Adult; Biological and medical sciences; Diagnosis, Differential; Encephalomyelitis - diagnosis; Encephalomyelitis - pathology; Female; Hematologic and hematopoietic diseases; Histiocytosis, Non-Langerhans-Cell - diagnosis; Histiocytosis, Non-Langerhans-Cell - pathology; Humans; intravascular lymphomatosis; Kidney Neoplasms - blood supply; Kidney Neoplasms - diagnosis; Kidney Neoplasms - pathology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Large B-Cell, Diffuse - diagnosis; Lymphoma, Large B-Cell, Diffuse - pathology; Medical sciences; reactive haemophagocytic syndrome; renal biopsy; Tropical medicine; Human; Immunohistochemistry; Nervous system diseases; Encephalomyelitis; Intravascular space; Malignant hemopathy; Large cell lymphoma; Histiocytosis; Non Hodgkin lymphoma; Kidney; Cerebral disorder; Case study; Pathology; Hemophagocytic syndrome; Biopsy; Lymphoproliferative syndrome; Central nervous system disease; Young adult; Female; Spinal cord disease
• ISSN: 0309-0167; 1365-2559
• DOI: 10.1046/j.1365-2559.1997.3300902.x

Aims: To report an unusual instance of intravascular lymphomatosis presented with encephalomyelitis and reactive haemophagocytic syndrome. There was no skin involvement. The diagnosis was made on a renal biopsy. Methods and results: The marrow smear was air dried and stained with Diff‐Quik. The tissue sections were stained with haematoxylin and eosin, Masson trichrome, periodic acid–Schiff's reagent, Elastic van Gieson's stain, modified hexamine‐silver technique and Martius scarlet blue. Immunohistochemistry for CD45, CD20, CD45RO, Factor VIII related antigen, CD31 and CD34 was performed on paraffin‐processed tissue. The marrow smear showed active haemophagocytosis in the histiocytes. The renal biopsy showed intravascular lymphomatosis with tumour cells positive for CD45 and CD20. Conclusion: The possibility of intravascular lymphomatosis should be considered in patients with reactive haemophagocytic syndrome where the underlying cause cannot be found after thorough investigation.