Intravascular large B-cell lymphoma associated with hypoalbuminemia and hypoxemia

A 67-year-old man was referred to our hospital for treatment of hemophagocytic syndrome. Hypotension, hypoxemia, pleural effusion, severe anasarca, and splenomegaly were noticed at the time of admission. Laboratory findings showed anemia (7.7 g/dl), thrombocytopenia (4.5 x 10(4)/microliter), an increase of serum LDH (1,466 IU/L) and severe hypoalbuminemia (1.9 g/dl). Bone marrow aspiration revealed an increase of reticulum cells with active hemophagocytosis and the presence of immature lymphocytes (6.0%). Lymphoma was suspected, but effective chemotherapy could not be performed because of progressive hypoxemia and severe hypoalbuminemia, and the patient died of the disease 2 weeks after admission. Autopsy revealed large lymphoid cells packed within systemic vessels as well as invasion into organs such as the liver, lungs, and spleen. The postmortem diagnosis was intravascular large B-cell lymphoma. Hypoalbuminemia and hypoxemia appear to be important clinical features of intravascular large B-cell lymphoma.     

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 large B-cell lymphoma with preceding syndrome of inappropriate secretion of antidiuretic hormone

Intravascular large B-cell lymphoma (IVL) is a rare subtype of B-cell lymphoma presenting with neurological and dermatological lesions in addition to generalized symptoms such as fever and malaise. It may also be associated with variable manifestations of affected organs due to extranodal progression predominantly in the lumen of the small vessels. Here, we report a case of IVL with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) as the sole manifestation at the initial presentation. The present case suggests that hormonal disturbances may progress in advance in IVL, before generalized symptoms develop.     

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.     

Primary mediastinal large B-cell lymphoma

Primary mediastinal large B-cell lymphoma (PMLBCL) is a unique type of B-cell lymphoma probably arising from a putative thymic medulla B-cell. It constitutes 6-10% of all diffuse large B-cell lymphomas (DLBCL), occurring more often in young females. PMLBCL is characterized by a diffuse proliferation of medium to large B-cells associated with sclerosis and a degree of compartmentalisation. Its main molecular characteristics include: gains in 9p segments, p53 mutations, BCL-2 and MAL gene over-expression, somatic mutations of IgVH genes, BCL-6, PIM-1, PAX-5, RhoH/TTF, and c-MYC, and constitutional NF-kappaB activation. The gene expression signature of PMLBCL seems to be much closer to classic Hodgkin lymphoma than to DLBCL. PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome. Most PMLBCL patients have stage I-II, bulky disease, with pleural or pericardial effusions in a third of cases. Systemic symptoms, mainly fever or weight loss, are present in <20% of cases; increased LDH levels are observed in 70-80% of cases. Treatment with CHOP regimen followed by radiation therapy was associated with a 5-year survival of 65%. Apparently better results have been reported with third-generation weekly alternating regimens followed by radiation therapy. Any recurrence is almost always seen in the first 2 years of follow-up, and distant relapses tend to involve extranodal organs. Features associated with poor prognosis are poor performance status, pericardial effusion, bulky disease, high serum LDH at diagnosis, and a compromised dose-intensity of anthracycline and cyclophosphamide.     

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.     

Intravascular B-cell lymphoma with hypercalcemia as the initial presentation

Intravascular B-cell lymphoma (IVLBCL) is a rare subtype of extranodal diffuse large B-cell lymphoma, which is characterized by the growth of lymphoma cells within blood vessel lumina without nodular lesions, and which predominantly affects elderly patients. IVLBCL is characterized by B-symptoms and a variety of systemic symptoms due to focal obstruction of blood flow, but may be difficult to diagnose due to its peculiar intravascular localization and the lack of nodular lesions. While hypercalcemia is one of the complications of various types of cancerous diseases, it has rarely been reported as the first presentation of IVLBCL. In this report, we present the case of a 71-year-old male with IVLBCL who showed hypercalcemia accompanied by elevation of serum parathyroid hormone-related protein (PTH-rP) as the initial presentation. Interestingly, immunohistochemical staining revealed that the intravascular lymphoma cells expressed high levels of PTHrP. Six courses of immunochemotherapy, consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP), and two courses of high-dose methotrexate induced complete remission (CR) and retained CR for 4 months. We also reviewed other IVBCL cases in which hypercalcemia was the initial presentation. We suggest that IVLBCL, although rare, should be considered as a possible causative in hypercalcemia of unknown underlying disease.     

Treatment of diffuse large B-cell lymphoma

A review of new or emerging ideas concerning diffuse large B-cell lymphomas (DLBCL) is presented with particular emphasis on histologic classification, genetic prognostic factors, and first-line treatments. This lymphoma rests the most heterogeneous of all lymphomas for its clinical characteristics and outcome. This heterogeneity is probably secondary to the fact that a large proportion seems to occur from a transformation of an unknown indolent lymphoma. Current treatment is based on the combination of chemotherapy including cyclophosphamide, adriamycin, vincristine, prednisone (CHOP) or dose-intense CHOP-like regimen, and rituximab (R-CHOP).     

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.