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 or intravascular lymphomatosis: report of a case diagnosed by testicle biopsy

Intravascular large B-cell lymphoma or intravascular lymphomatosis (IVL) is an extremely rare form of non-Hodgkin's lymphoma. The most common clinical sign is fever of unknown origin (FUO). Histologically, there is proliferation of malignant lymphoid cells within vascular lumina. Cytologically, the cells have features similar to those found in classical large cell lymphoma. Examination of pulmonary artery blood showed the presence of this abnormal population in our patient; to the best of our knowledge there are only four other. reports of detection of circulating tumor cells in IVL. The outcome is very poor. The diagnosis is most frequently made after biopsy of skin or brain but is often established post mortem. We present what is--to our knowledge--the first reported case of IVL diagnosed after biopsy of a testicle. In the event of FUO and suspicion of a malignancy, IVL--although very rare--should be one of the differential diagnoses.     

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.     

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.     

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 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.     

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 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.     

Intravascular lymphomatosis manifesting in the lung

A case of intravascular lymphomatosis with predominant symptoms in the lung (cough and interstitial pulmonary infiltrates) is presented. Fever and confusion were lateness symptoms. Examination postmortem established the diagnosis. In the lung alveolar walls, small arterioles and the capillaries were occupied by atypical cells positives for CD45 and CD20, leukocyte and pan-B markers, and negatives for CD45-Ro and CD34, pan-T and endothelial markers. The main characteristics and the lung participation of this entity were reviewed.     

骨髓免疫组织化学染色确诊神经母细胞瘤1例

介绍1例神经母细胞瘤（neuroblastoma,NB）骨髓转移的确诊方法。通过骨髓细胞形态学和细胞免疫组织化学染色,判断骨髓转移瘤细胞种类,以协助诊断原发肿瘤。确诊转移瘤细胞为NB细胞。骨髓细胞形态学加细胞免疫组织化学染色检查,可协助确诊骨髓转移瘤的类型。     

Intravascular lymphomatosis. A report of three cases

Intravascular lymphomatosis (IVL) is a rare malignancy characterized by a proliferation of atypical lymphoid cells occluding small blood vessels (venules, capillaries and small arteries). The symptoms are caused by embolisms due to massive proliferation. Nervous system and skin are the most common sites of involvement but all organs may be involved, although it is typified by the absence of malignant cells in lymphoid tissues. We describe three cases of IVL: first patient was a 57 years old man with rapidly progressive dementia and neurological involvements and second case of a 69 years old man hospitalised with predominant symptoms in the lung (cough, dyspnea and fever), and a woman presenting as fever of unknown origin (FUO) with systemic inflammatory response syndrome. In all cases that we reported have been diagnosed post-mortem because of rapid progression of a multisystem disease and the absence of pathognomonic clinical manifestations. Diagnosis can be made using biopsy of one of the involved organs. In conclusion, we propose that IVL should be included in the differential diagnosis of acute confusional state, dementia or other unexplained neurological manifestations, fever of unknown origin, vasculitis, occult neoplasia or infections with signs of a systemic disease and marked elevation of serum lactate dehydrogenase (LDH).     

Intravascular lymphomatosis presenting as systemic inflammatory response syndrome

Intravascular lymphomatosis is an unusual form of non-Hodgkin lymphoma characterized by intravascular proliferation of atypical lymphoid cells in multiple organs. It can cause systemic inflammatory response syndrome due to primary release of cytokines by the tumor cells or secondary release of cytokines after vascular occlusion by the tumor cells. It is a potentially fatal condition, because multiorgan failure can ensue due to thrombotic vascular occlusion. This is a very rare condition and most cases are diagnosed post mortem. We present a case of systemic inflammatory response syndrome and subsequent death from multiorgan failure in a patient with intravascular lymphoma.     

Intravascular lymphomatosis presenting as adult respiratory distress syndrome

An unusual case of intravascular lymphomatosis caused by small noncleaved, non-Burkitt's lymphoma, which presented with adult respiratory distress syndrome, is described. Extensive invasion of the small- and medium-size blood vessels of the lung, liver, spleen, kidneys, heart, esophagus, stomach, small and large intestines, bladder, and brain—but not the bone marrow or peripheral blood—is documented. The possible mechanism and the unusual features of this case are discussed in comparison with previously reported cases. The pertinent literature is reviewed. The problem of diagnosing this pathological entity is emphasized. Am. J. Hematol. 56:155–160, 1997. © 1997 Wiley-Liss, Inc.