Successful treatment of intravascular malignant lymphomatosis with high-dose chemotherapy and autologous peripheral blood stem cell transplantation

Abstract

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...

Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions

“…3 At the time of analysis, the combination of rituximab and chemotherapy has been reported to have been used in 15 patients (six in our series), resulting in complete remission in 11 of 12 evaluable patients, and no relapses at a median followup of 15 months; this combined strategy seems to be advisable in patients with CD20-positive IVL. Consolidation with high-dose chemotherapy supported by autologous stem cell transplantation may improve current outcomes, [21][22][23] even among J-HPC patients. 9 The application of such a strategy is, however, greatly limited by the high median age and poor PS of IVL patients.…”

Intravascular Lymphoma: The Oncologist's “Great Imitator”

“…Interestingly, two cases report sustained remissions after monotherapy with rituximab [43,44]. There are now seven case reports of long-term survival after autologous hematopoietic stem cell transplantation: three patients in first CR (one with CNS involvement) [40,45]; four in second CR [46][47][48][49]; and two patients in whom bone marrow transplant in second CR was unsuccessful [40].…”

Long-term remission after high-dose chemotherapy followed by auto-SCT as consolidation for intravascular large B-cell lymphoma

“…[2][3][4] High-dose chemotherapy (HDC) followed by autologous PBSC transplantation (auto-PBSCT) might be an efficient treatment; however, there are insufficient data to support the efficacy of HDC/auto-PBSCT against IVLBCL. 2,5,6 A recent report demonstrated that up-front HDC/ auto-PBSCT improved the 2-year PFS to 69% in patients with international prognostic index (IPI) high/intermediate or high-risk DLBCL and responses to induction therapy, in comparison to the 2-year PFS of 55% in patients with induction therapy alone. 7 As most patients with IVLBCL were classified into the IPI high-risk group, the up-front HDC/auto-PBSCT during the first CR may represent a useful treatment option for patients with IVLBCL.…”