Intravascular Large B-Cell Lymphoma

Abstract

A rare type of diffuse large B-cell lymphoma, intravascular large B-cell lymphoma primarily affects the middle-aged to elderly population, with a slight predominance in men. By the time of presentation, most patients have advanced, disseminated disease, and often the diagnosis is made at autopsy. Patients may present with any of a myriad of symptoms, with any tissue potentially being infiltrated. Central nervous system and cutaneous involvement is common, as is the presence of B symptoms including fever, weight loss, and night sweats. Morphologically, growth of neoplastic cells is restricted to the lumen of small vessels. The cells are large, with 1 or more prominent nucleoli, scant cytoplasm, and frequent mitotic figures, and are commonly positive for cluster of differentiation markers 79a, 20, and 19, as well as MUM1/IRF4 and Bcl-2. Intravascular large B-cell lymphoma is aggressive, and without treatment is rapidly fatal.

Intravascular Large B-Cell Lymphoma: A Continuing Clinical Enigma

“…12 Symptoms secondary to CNS involvement are common and varied and may range from sensory and motor deficits, altered conscious state, paresthesias to seizures. 13 Quite unusual in this case is the presence of the neoplastic cells confined in the small blood vessels of the uterus and cervix. Although it has been frequently mentioned in different studies that IVLBCL can virtually involve any organ, the infiltration of the blood vessels in the gynecologic tract is rarely noted.…”

Isolated Intravascular Large B-cell Lymphoma Presenting with Diffuse Ground Glass Pulmonary Infiltrates

“…The patient was subsequently started on cyclophosphamide, doxorubicin, vincristine, and prednisone and completed eight cycles without clinical or radiologic signs from whole body positron emission tomography-computed tomography (PET-CT) of tumor recurrence or metastasis. IVLBCL is an extremely rare malignancy with an approximate incidence of less than one per million per year [1]. In 2008, the World Health Organization (WHO) classified IVLBCL as a type of extranodal large B-cell lymphoma.…”

“…In 2008, the World Health Organization (WHO) classified IVLBCL as a type of extranodal large B-cell lymphoma. IVLBCL is characterized by proliferation of lymphoma cells within the lumen of small blood vessels, especially capillaries [1,2]. More than 90 % of cases present as clinical stage III or stage IV disease and can be rapidly fatal if diagnosis and treatment are delayed.…”

“…More than 90 % of cases present as clinical stage III or stage IV disease and can be rapidly fatal if diagnosis and treatment are delayed. However, current chemotherapy regimens including rituximab have resulted in improved survival with the possibility of prolonged remission as was seen in our case [1]. Diagnosis of IVLBCL can be challenging given its nonspecific presentation and possibility for involvement of virtually any organ given its hematogenous nature [2][3][4].…”

“…Anemia, leukopenia, thrombocytopenia and elevated levels of CRP, LDH, ferritin, IL-2R and ß2 microglobin are often noted. Patients with pulmonary involvement can present with cough, dyspnea, hypoxemia and pulmonary hypertension with chest CT showing focal nodules, diffuse ground glass infiltrates or consolidation [1]. A biopsy is mandatory for the diagnosis and shows large malignant lymphoid cells (CD 20 positive, CD3 negative) in small vessels and capillaries with prominent nucleoli, frequent mitotic figures and scant cytoplasm [1].…”