Intravascular malignant lymphomatosis diagnosed on a muscular biopsy: a case report

Intravascular malignant lymphomatosis (IML) is characterized by proliferation of malignant lymphoid B cells within the lumens of small vessels. Common symptoms include general weakness and central neurological and cutaneous signs. Only histopathological analysis can confirm the diagnosis. We report on a 69-year-old man hospitalized for general weakness, inflammatory syndrome, and hemophagocytic syndrome (HS). Our observation shows that histopathological signs may be observed on a muscular biopsy without clinical or biological signs of muscular involvement.     

Intravascular large B-cell lymphoma diagnosed by FDG-PET/CT and endometrial biopsy

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin's lymphoma characterized by a proliferation of tumor cells within the lumina of small to medium-sized vessels. Because there are few or no concomitant solid lesions, a diagnosis of IVLBCL usually cannot be established by CT or MR imaging. Herein, we describe a case of IVLBCL involving the uterus, in which (18)F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) was useful for diagnosis. A 47-year-old woman was referred to our hospital because of fever and anemia. Laboratory examination demonstrated anemia and thrombocytopenia. Bone marrow aspiration and biopsy showed hemophagocytosis without involvement of lymphoma cells. Random skin biopsy did not demonstrate lymphoma involvement. FDG-PET/CT imaging showed FDG accumulation in the uterus. MR imaging demonstrated uterine leiomyoma only. Based on these findings, uterine endometrial biopsy was performed and histological diagnosis of IVLBCL involving the uterus was established. She received 6 courses of R-CHOP therapy and high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. At present, she remains in complete remission after 33 months.     

Asian variant of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy

A 63-year-old male presented with fever and general malaise in June 2004. On admission hepatosplenomegaly was apparent, but without lymphadenopathy. The laboratory examination revealed pancytopenia and increased levels of lactate dehydrogenase, direct bilirubin and soluble interleukin-2 receptor. Histological analysis of the bone marrow biopsy specimen demonstrated proliferation of atypical lymphoid cells positive for CD20 in the small capillaries, leading to the diagnosis of the Asian variant of intravascular large B-cell lymphoma (AIVL). The presence of rearrangement of the immunoglobulin gene confirmed the diagnosis. The patient responded well to CHOP therapy followed by seven courses of rituximab-combined CHOP therapy and has remained in complete remission up to the present. This case implies that bone marrow biopsy could be a useful examination for diagnosing AIVL and that rituximab-combinedchemotherapy could improve survival in patients with the disease.     

Intravascular lymphomatosis diagnosed by bone marrow biopsy

Intravascular lymphomatosis (IVL) is a rare malignancy characterized by neoplastic proliferation of lymphoid cells within the lumina of small vessels. We report a case of IVL in a 69-year-old woman, who presented with pancytopenia and elevation of the serum LDH level. There was no skin eruption or neurological abnormalities. Clusters of abnormal lymphoid cells were barely evident in a peripheral blood smear. Laboratory examinations revealed high levels of LDH (2,602 IU/l) and sIL-2R (5,640 U/ml). Bone marrow aspiration revealed a normal cellular marrow with mild hemophagocytosis, but no tumor cells were detected. After admission, respiratory failure due to multiple pulmonary embolisms progressed, and continuous infusion of heparin had no apparent effect. Bone marrow vessels filled with lymphoma cells were observed in a biopsy specimen, thus establishing a diagnosis of IVL. Chemotherapy with the CHOP regimen was immediately instituted. The respiratory failure was dramatically improved, resulting in disappearance of the abnormal lymphoid cells from the bone marrow. After eight courses of CHOP, low-dose etoposide therapy was administered, and no symptoms of relapse were noticed. The diagnosis of IVL is difficult because it does not form masses of tumor cells. Bone marrow biopsy may be helpful for early diagnosis of IVL if the disease is suspected and searched for.     

A case of pulmonary intravascular lymphomatosis diagnosed by thoracoscopic lung biopsy

Intravascular lymphomatosis with primary pulmonary lesion is an extremely rare disease. Although the major clinical symptoms include fever, cough, dyspnea and loss of body weight, these are not diagnostic. Chest radiograph findings are also nonspecific and include bilateral reticular shadow, reticulonodular shadow, ground-glass opacity or wedge-shaped subpleural opacities. Therefore, the antemortem diagnosis is relatively difficult. It is considered that intravascular lymphomatosis is a high-grade malignant lymphoma. However, it has been shown recently that a good response and long-term survival may possibly be obtained through systemic combination chemotherapy. We report a case of intravascular lymphomatosis with primary pulmonary lesion where an early diagnosis was obtained through thoracoscopic lung biopsy and subsequent systemic chemotherapy proved to be quite effective. Because the clinical symptoms or chest radiograph findings are usually nonspecific, it was thought that thoracoscopic lung biopsy could be a useful procedure for early and reliable diagnosis of primary pulmonary intravascular lymphomatosis and that it might contribute to an improved prognosis.     

Successful treatment of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy and FDG-PET scan

Early diagnosis of intravascular large B-cell lymphoma (IVLBCL) is difficult, but is critical for longer survival for the patients. We report a case of IVLBCL that was diagnosed with the help of FDG-PET. A 76-year-old woman was referred to us for the evaluation of her elevated serum LDH. She presented with general malaise and high fever. There were no skin lesions or neurological involvement. FDG-PET imaging showed increased uptake of FDG in the vertebra, bilateral femurs, sternum, and iliac bones. A diagnosis of IVLBCL was made by bone marrow biopsy. She was successfully treated with rituximab and modified CHOP therapy.     

Intravascular large B cell lymphoma diagnosed by senile angioma biopsy

An 82-year-old man without notable medical history was admitted to our hospital following subacute deterioration of apettite, disorientation and strange behavior. There was spasticity of the right extremities without weakness. LDH and serum soluble IL2 receptor antibody levels were elevated, and as well as the protein level and IgG levels in the cerebrospinal fluid. CT scanning of the brain revealed a lesion at the left corona radiata. The patient's level of consciousness was worsening, and follow-up study of the brain showed a new lesion in the left occipital lobe. Intravascular lymphomatosis was therefore suspected. We performed a skin biopsy from two typical senile angiomas. In one of these biopsy specimens, a capillary hemangioma was present in the mid-reticular dermis and it was filled with abnormal B cells. The diagnosis of intravascular B cell lymphoma (IVL) was thus established. IVL is a rare subtype of extranodal diffuse large B cell lymphoma with a poor outcome. However, it is recently thought that if the diagnosis is established early, aggressive chemotherapy increases survival. Senile angioma is a skin eruption that is considered prevalent for the most part in elderly people. If a patient is suspected to have IVL, and there is no appropriate site of biopsy, it might be beneficial to try a skin biopsy aiming at senile angiomas for early diagnosis.     

自体造血干细胞移植治疗血管内大B细胞淋巴瘤1例

目的探讨血管内大B细胞淋巴瘤（IVLBCL）的诊治。方法报告经自体造血干细胞移植治疗的1例女性IVLBCL患者的诊治经过,并复习相关文献。结果患者经肺活检及肾脏活检病理诊断为IVLBCL。经过R-CHOP方案6个疗程治疗后取得完全缓解,再经R-CHOPE方案1个疗程并动员采集自体外周血干细胞,行R-CBV方案为预处理方案的自体外周血干细胞移植,之后用利妥昔单抗巩固及维持治疗。术后随访25个月,患者持续完全缓解状态。结论本例IVLBCL患者采用R-CHOP联合自体外周血干细胞移植治疗及利妥昔单抗巩固维持取得满意疗效。     

Primary intravascular large B-cell lymphoma of the lung: a review and case report

Objective

To investigate the clinicopathological features of primary intravascular large B-cell lymphoma (IVLBCL) of the lung.

Methods

Histopathological and clinical data based on lung biopsy were analyzed and used to diagnose a patient with IVLBCL of the lung.

Results

Fever and respiratory symptoms were the main presentations, lung biopsy revealed lymphoma cells in the lumen of small blood vessels. Tumor cells expressed Bcl-2, the Bcl-6, CD20, Ki67, MUM-1, Pax5, CD, CD30, and vascular endothelial CD34.

Conclusions

Primary pulmonary IVLBCL of the lung is extremely rare, on chest CT it manifests as diffuse ground glass shadow, or nodular consolidations in the lung, lactate dehydrogenase and C-reactive protein was found to increase, fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) is an important and significant diagnostic modality in its early diagnosis. Also, bronchial lung biopsy has the advantage of less trauma and high sensitive rate. R-CHOP is the main treatment for lung primary pulmonary IVLBCL of the lung; however, its prognosis is relatively poor.     

Intravascular B-cell lymphoma in a 38-year-old woman: a case report

Intravascular lymphoma (IVL) is a rare aggressive disease characterised by the presence of lymphoma cells only in the lumina of small vessels, particularly capillaries. Only about 200 cases have been reported in the world (some of them retrospectively). IVL is predominantly of B-cell lineage origin but occasionally T-cell lineage occurs. Multiple organs may be involved and a variety of clinical presentations have been described. These include nephrotic syndrome, pyrexia and hypertension, breathlessness and haemolytic anaemia, leukopoenia, pancytopoenia and disseminated intravascular coagulation. We report a case of a 38-year-old woman with a highly aggressive clinical course of IVL. She was admitted to the Department of Neurosurgery because of spondylolisthesis of L5-S1 qualified to surgery. During hospitalisation haemolytic anaemia, thrombocytopoenia and splenomegaly were observed and she was admitted to the Department of Haematology for diagnosis. During her staying in the hospital, new symptoms, such as kidney and liver failure, occurred and the central nervous system was involved. The clinical course was very rapid and progressive. Corticosteroid therapy was started but the disease soon led to the fatal outcome. Diagnosis was established at post-mortem examination.     

Two cases of intravascular lymphomatosis diagnosed antemortem by transbronchial lung biopsy]

Patient 1 was a 71-year-old man who had been admitted to our hospital with high-grade fever. Chest computed tomographic (CT) images revealed clear peripheral and high-density central areas. Abnormal laboratory findings included elevated LDH and hypoxemia. Interstitial pneumonia was suspected, and transbronchial lung biopsy (TBLB) confirmed the diagnosis of intravascular lymphomatosis (IVL). The patient succumbed before completion of chemotherapy. Patient 2 was a 65-year-old man admitted with high-grade fever. Abnormal laboratory findings included pancytopenia, hypoxemia, and elevated levels of LDH and soluble interleukin-2 receptor. Chest CT images revealed diffuse, mildly dense areas in the upper fields of both lungs. TBLB specimens yielded a diagnosis of IVL. Complete clinical remission was obtained with CHOP multiagent chemotherapy. Although IVL is usually diagnosed at autopsy, in these 2 cases an antemortem diagnosis was made on the basis of TBLB findings. Also, multiagent chemotherapy achieved a complete clinical remission in Patient 2.     

Intravascular large B-cell lymphoma with involvement of the abdominal subcutis: a case report and literature review

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma (DLBCL). It commonly presents with a variety of symptoms due to occlusion of small vessels by tumor cells in different organ systems. Clinically patients may present with generalized symptoms such as fever and malaise. In western patients, there is a ‘cutaneous variant’ of IVLBCL, which demonstrates cutaneous involvement only. However, Asian patients show hemophagocytosis, which is typical of the ‘Asian variant’. Here we report a case of IVLBCL in a Chinese individual who presented with a huge mass in the subcutis of the abdomen. Wenjuan Yin, Min Li are contributed equally to this work.     

Intravascular large B-cell lymphoma presenting as rapidly progressive dementia and stroke: A case report

Rationale:Intravascular large B-cell lymphoma (IVLBCL) is a rare form of large B-cell non-Hodgkin lymphoma. The diagnosis is challenging and frequently made at biopsy. Here we reported a case of IVLBCL limited to the central nervous system (CNS) presenting with progressive dementia and acute stroke, who was diagnosed by brain biopsy.Patient concerns:A 47-year-old woman was transferred to our hospital with a 6-month history of rapidly progressive dementia, and left limb weakness and numbness for 3 days. She was successively misdiagnosed with inflammatory demyelinating disease and stroke. Her condition deteriorated with elevated lactate dehydrogenase and multiple hyperintense lesions on the brain.Diagnosis:She was diagnosed with IVLBCL limited to the CNS by brain biopsy.Interventions:Bone marrow puncture and incisional random skin biopsy were not found neoplastic cells. Computed tomography scans were normal with no evidence of disease outside the CNS.Outcomes:The patient died due to rapid clinical aggravation.Lessons:IVLBCL limited to the CNS is an aggressive disease with high mortality. Making a timely and correct diagnosis is crucial for early appropriate treatment in IVLBCL patients.     

Intravascular lymphomatosis mimicking acute disseminated encephalomyelitis: a case report

We describe the clinical, radiologic, and postmortem findings in a 49-year-old woman with intravascular lymphomatosis. The patient presented with progressive limb weakness followed by progressive disseminated cerebral neurologic symptoms. Disseminated encephalomyelitis was suspected due to the clinical and radiologic findings. Steroid pulse therapy and intravenous immunoglobulin were given but did not help. The patient died of multiple organ failure 3 months after the onset of symptoms. A diagnosis of disseminated intravascular large B-cell lymphomatosis was established based on findings from histopathology and immunohistochemistry studies on autopsy specimens of the brain and other visceral organs collected postmortem.     

A case of intravascular large B-cell lymphoma (IVLBCL) with no abnormal findings on chest computed tomography diagnosed by random transbronchial lung biopsy

A 58-year-old woman was admitted with refractory fever despite receiving broad-spectrum antibiotics. She had hypoxemia, severe anemia, elevated levels of serum lactic dehydrogenase and soluble interleukin-2 receptor, and a positive direct Coombs test, which suggested an underlying autoimmune hemolytic anemia (AIHA). Chest computed tomography (CT) showed no abnormal findings, but she had hypoxia, and her alveolar-arterial oxygen difference (A-aDO2) was increased. A random transbronchial lung biopsy (TBLB) was performed, and pathological analysis showed massive proliferation of tumor cells in the lumina of the small vessels. Intravascular large B-cell lymphoma (IVLBCL) was diagnosed, and her general status improved after chemotherapy.     

Clinical course of 8 patients with intravascular large B-cell lymphoma diagnosed while alive

We retrospectively evaluated the diagnosis and clinical courses of 8 patients with intravascular large B-cell lymphoma (IVL) diagnosed while they were alive. The median age was 67 years old (range 54 to 82). Most complaints at diagnosis were fever or dyspnea. All patients were in clinical stage IV with B symptoms and 4 patients showed performance status 4. The diagnosis of IVL was confirmed by biopsy specimens from the bone marrow in 4, lung in 2, muscle, adrenal gland, and lymph node in 1 case, respectively. Initial bone marrow involvement was found in 6 patients. Chemotherapy was performed in 7 patients. Rituximab was added to chemotherapy in 5 patients. Though 5 patients are alive at the median follow up of 12.3 months, only 1 patient is in remission. Four of 5 patients treated with Rituximab relapsed. In suspicious cases, it is important to bear IVL in mind and examine bone marrow biopsies for an early diagnosis. In addition, it is suggested that Rituximab may play only a temporary role in the treatment of IVL.     

Intravascular B-cell lymphoma with leukemic presentation: case report and literature review

Intravascular lymphoma is an extremely rare, disseminated, and aggressive extranodal CD20⁺ non-Hodgkin's lymphoma characterized by the presence of lymphoma cells only in the lumina of small vessels. We report a 72-year-old woman with a diagnosis of intravascular lymphoma presented with splenomegaly and leukemic appearance in the peripheral blood smear. Her clinical course was rapidly deteriorated before the initiation of specific chemotherapy and finally died due to multiorgan insufficiency. Bone marrow biopsy revealed a characteristic infiltration of CD5, CD10 B-cell lymphoma. To our knowledge, this is the first reported case of a CD5, CD10 intravascular B-cell lymphoma with leukemic presentation in peripheral blood with multiple cytogenetic aberrations.