Summary:Intravascular malignant lymphocytosis (IML) is a rare systemic disease characterized by proliferation of malignant B (rarely T) lymphoid cells within the lumina of small arteries, veins, and capillaries. Diagnosis requires skin, liver, renal, meningeal, or brain biopsy, but is rarely made ante mortem. In this report, we describe a patient who had an ante mortem diagnosis of IML as a result of a skin biopsy. Autologous peripheral blood stem cell transplantation (auto-PBSCT) was successfully performed after chemotherapy. The patient has survived for more than 30 months since the onset of the disease and maintains complete remission on the 450th day post PBSCT. To our knowledge, this is the first case of IML treated by auto-PBSCT. Bone Marrow Transplantation (2001) 27, 1101-1103. Keywords: intravascular malignant lymphocytosis (IML); skin biopsy; CHOP; auto-PBSCT
Case reportA 52-year-old man presented to our hospital in September 1998 with a 9-month history of progressive dementia, hemiparesia, urinary retention, and weight loss. Hasegwa dementia scale (HDS), that is the scale to evaluate the degree of dementia, was 12 point. He had reddish indurated macules scattered over the anterior chest surface. Routine hematological, biochemical and serological values were all within normal limits. Examination of bone marrow showed no abnormal cells. Magnetic resonance imaging (MRI) of the brain demonstrated an increased periventricular signal on T2-weighted images. Computed tomography (CT) scans of the brain disclosed low density areas in the corresponding section. Positron emission tomography of the brain showed degraded blood flow in the left temporal and frontal lobe and right cerebellum. Results of bone marrow biopsy, cerebrospinal fluid examination, CT scans of chest and abdomen, abdominal ultrasound sonography and whole body gallium scintigram were normal. A skin biopsy specimen of the anterior chest eruption revealed atypical hyperchromatic large lymphoid cells within the lumena of capillary vessels. These cells also infiltrated the extravascular spaces. Immunohistochemical study showed that these cells were positive for a B cell marker L26 (CD20), but not for a T cell marker MT-l (CD43) nor endothelial cell marker (CD34). DNA obtained from these cells and bone marrow cells at the time of lymphoma diagnosis showed no immunoglobulin gene rearrangements by reverse transcription polymerase chain reaction (RT-PCR). In addition, the serum soluble interleukin 2 receptor (s-IL2R) value was elevated to 1610 U/ml. Based on the results stated above a diagnosis of IML (B cell type) was established.The clinical course is shown in Figure 1. The patient was treated with a standard regimen comprising doxorubicin, cyclophosphamide, vincristine and prednisone (CHOP). Amnesia, hallucinations, dementia and skin macules regressed after one cycle and the patient achieved a complete response after three cycles, although urinary incontinence and hemiparesis remained. Positron emission tomography of the brain showed normal blood flow...