This study aimed to establish a standard treatment for disseminated extranodal large B-cell lymphoma, including intravascular large B-cell lymphoma (DEN-LBCL/IVL), and to validate the clinical diagnostic criteria we proposed. Between 2006 and 2016, 22 patients were enrolled in a clinical trial conducted by the Hokuriku Hematology Oncology Study Group. The first cycle of chemotherapy consisted of dose-reduced cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) with delayed administration of rituximab. From the second to the sixth cycle, patients received conventional rituximab and CHOP therapy. The primary endpoint was overall survival (OS), while the secondary endpoints included the complete response (CR) rate and time to treatment failure (TTF). The results showed a CR rate of 73%, a median OS of 65 months, and a median TTF of 45 months. These findings indicate that patients with DEN-LBCL/IVL were effectively treated with our new chemoimmunotherapy regimen. Our clinical diagnostic criteria are useful for identifying patients who require early intervention.

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Intravascular large B-cell lymphoma (IVLBCL), a rare subtype of malignant lymphoma, is diagnosed by observation of intravascular proliferation of tumor cells in samples taken from affected organs. However, diagnosis of IVLBCL is usually difficult due to the lack of mass formation. IVLBCL may be fatal when the diagnosis is delayed, so an accurate early diagnosis is the key to successful treatment. Random skin biopsy (RSB), in which specimens are sampled from normal-appearing skin, has been reported as useful. However, the specific method of RSB remains controversial, with individual institutions using either the punch method or the incisional method. Research has shown that the incisional method has higher sensitivity than the punch method. We discuss whether this difference might owe to the collection of punch specimens from an insufficient depth and whether the punch method might result in false negatives. For RSB, we recommend taking specimens not only from normal-appearing skin, but also from any lesional skin, because lesions may reflect micro IVLBCL lesions. To ensure accurate diagnosis, both dermatologists and hematologists should know the proper method of RSB. This review summarizes the appropriate biopsy method and sites for RSB.

UNASSIGNED: Intravascular large B-cell lymphoma (IVLBCL) is a rare entity among large B-cell non-Hodgkin lymphomas and is often difficult to diagnose. We report the case of a patient with IVLBCL who presented with central nervous system (CNS) symptoms alone, in which positron emission tomography (PET) enabled a rapid and accurate diagnosis.
UNASSIGNED: An 81-year-old woman was admitted to our hospital with a 3-month history of gradually progressive dementia and declining spontaneity. Magnetic resonance imaging revealed multiple hyperintense lesions bilaterally on diffusion-weighted imaging without enhancement on gadolinium-enhanced T1-weighted imaging. Laboratory findings showed elevated serum lactate dehydrogenase (626 U/L) and soluble interleukin-2 receptor (sIL-2R) (4692 U/mL). Cerebrospinal fluid (CSF) analysis showed slightly elevated levels of protein (166 mg/dL) and lymphocytic cells (29/μL), and β2-microglobulin (β2-MG) (4.6 mg/L) was highly elevated. Whole-body computed tomography revealed faint ground-glass opacities in the upper and middle lung fields and diffuse enlargement of both kidneys without lymph node swelling. 18F-fluorodeoxyglucose (FDG)-PET showed diffuse and remarkably high FDG uptake in both upper lungs and kidneys without uptake by lymph nodes, suggesting a malignant hematological disease. IVLBCL was confirmed histologically by incisional random skin biopsy from the abdomen. Chemotherapy using R-CHOP regimen in combination with intrathecal methotrexate injection was started on day 5 after admission and follow-up neuroimaging showed no signs of recurrence.
UNASSIGNED: IVLBCL presenting with CNS symptoms alone is rare and often has a poor prognosis associated with delayed diagnosis, and various evaluations (including systemic analysis) are therefore necessary for early diagnosis. FDG-PET, in addition to identification of clinical symptoms and evaluation of serum sIL-2R and CSF β2-MG, enables rapid therapeutic intervention in IVLBCL presenting with CNS symptoms.

Intravascular B-Cell Lymphoma (IVBCL) is a rare type of extranodal large B-cell lymphoma where clonal B-cells selectively home to and replicate in the lumen of small vessels. Random skin biopsy (RSB) of uninvolved skin is a reputable tool to diagnose this protean entity and thus dermatologists are involved in its diagnosis. A literature review was completed to derive an approach to RSB in these patients to maximize diagnostic yield of IVBCL and minimize morbidity. Based on this review, data from 27 patients where clinical signs and symptoms and results of investigations were able to be linked to a positive diagnosis of IVBCL from RSB from 11 papers from 2003 to 2021 were analyzed. Following this analysis, RSB should be considered when there are no clinical skin findings and there is an elevated lactate dehydrogenase in the absence of lymphadenopathy and presence of fever of unknown origin, thrombocytopenia, anemia, and ferritinemia. Three to four RSBs from the thigh, abdomen and/or posterior upper arm should be performed via either incisional or telescoping punch biopsies and should include senile/cherry angioma(s) if present. If RSB results in a diagnosis of IVBCL, hematology should be consulted for further management. Consideration of a false negative biopsy or alternative diagnoses should be explored if RSB is negative for IVBCL. Following a standard approach for RSB in these patients will increase diagnostic yield of IVBCL while decreasing the risk of harm to the patient.

UNASSIGNED: Delayed diagnosis of intravascular large B-cell lymphoma (IVLBCL) is associated with a poor prognosis, making early diagnosis and treatment essential. However, early diagnosis remains challenging.
UNASSIGNED: Here we present the case of a 75-year-old man with fever of unknown origin, in whom random skin biopsy (RSB) allowed early diagnosis of IVLBCL.
UNASSIGNED: The usefulness of RSB, which involves incisional skin biopsies of three or more sites that contain subcutaneous fatty tissue, such as the thighs, abdomen and upper arms, has been debated. In cases of suspected IVLBCL, RSB is less invasive than a biopsy of the internal organs.
UNASSIGNED: We suggest that combining RSB with bone marrow examination may facilitate the diagnosis of IVLBCL.
CONCLUSIONS: Delayed diagnosis of intravascular large B-cell lymphoma (IVLBCL) is associated with a poor prognosis, but early diagnosis is difficult due to its diverse, non-specific presentation.This case report illustrates the value of random skin biopsy in the early diagnosis of IVLBCL in a man with fever of unknown origin and haematological abnormalities without lymphadenopathy, splenomegaly or bone marrow abnormalities.The diagnostic accuracy of random skin biopsy depends on several factors, including the location and number of sites biopsied, and the depth and width of the biopsy.

A 79-year-old man was admitted to our hospital because of sudden onset of left ataxic hemiparesis. Brain MRI diffusion weighted images showed typical lacunar infarction on the right internal capsule. He had no risk factors of cerebrovascular disorder such as hypertension, diabetes mellitus, hyperlipidemia and arrhythmia. On admission, he had a slight fever and his laboratory data showed anemia, thrombocytopenia and elevation of CRP and LDH. Intravascular large B-cell lymphoma (IVLBCL) was suspected because the serum level of soluble IL-2 receptor was also elevated. Pathological diagnosis of IVLBCL was underwent by the skin biopsy from his senile hemangiomas. Although IVLBCL was known to demonstrate various CNS lesions, it is extremely rare to be manifested as a single lacunar infarction, and this case must be important for the differential diagnosis.

Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoma characterized by the selective growth of lymphoma cells within the lumen of vessels. We describe the case of a 69-year-old male who presented with marked pain in the left facial region. Gadolinium-enhanced magnetic resonance imaging revealed a swollen left trigeminal nerve (TN) and positron emission tomography/computed tomography demonstrated fluorodeoxyglucose-only uptake at the same site. The patient had high serum lactate dehydrogenase and soluble interleukin-2 receptor levels. As random skin biopsy and bone marrow biopsy detected no abnormal pathogenesis, open biopsy of the TN was performed, revealing diffuse large B-cell lymphoma (DLBCL). However, ground glass opacities rapidly developed in both lung fields with severe respiratory failure. The patient died of progressive disease before the initiation of chemotherapy. Postmortem examination revealed widespread lymphoma cells in the lumen of vessels in multiple organs, including the lungs, excluding the bone marrow and skin. Lymphoma cells formed a mass in the TN and left lumbar plexus. A diagnosis of IVLBCL was made based on the postmortem pathological analysis. DLBCL of abnormal sites, such as the peripheral nervous system, should be considered in cases of IVLBCL as a differential diagnosis.

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of diffuse LBCL. The patient was a 71-year-old female admitted to our hospital with hypoxia. On admission, chest computed tomography revealed a ground-glass opacity. Interstitial pneumonia associated with systemic scleroderma was suspected because of positive anti-centromere antibody. Thereafter, steroid pulse therapy and plasma exchange were performed. Although ground-glass opacity improved, bilateral pleural effusion appeared, so we performed a random skin biopsy because of her elevated serum lactate dehydrogenase and soluble interleukin-2 receptor levels. The patient was diagnosed with IVLBCL with symptoms improving after 6 cycles of rituximab plus chemotherapy treatment.

BACKGROUND: Intravascular lymphoma (IVL) is a rare subtype of lymphoma involving the growth of lymphoma cells within the vessel lumina without lymphadenopathy. Because of various modes of presentation and its rarity, IVL is often diagnosed postmortem. Herein, we report a case of intravascular B-cell lymphoma with hypopituitarism, an extremely rare complication, that was successfully treated with chemotherapy.
METHODS: An 80-year-old Japanese woman presented with a 7-mo history of a tingling sensation in the lower limbs. She also presented with various other symptoms such as pancytopenia, high fever daily, and unconsciousness with hypoglycemia. Although the doctor who previously treated her diagnosed hypoglycemia as being due to hypopituitarism, the cause of the other symptoms remained uncertain despite a 7-mo evaluation period. We performed bone marrow aspiration to evaluate pancytopenia and found that she had hemophagocytic lymphohistiocytosis (HLH). On the basis of a random skin biopsy for assessing the cause of HLH, she was diagnosed with intravascular B-cell lymphoma. HLH and hypopituitarism were considered secondary to IVL. All her clinical findings matched the presentations of IVL. She was immediately treated with chemotherapy and achieved complete response. She was relapse free two years after treatment.
CONCLUSIONS: IVL should be included in the differential diagnosis of hypopituitarism, which although life-threatening, is treatable through prompt diagnosis and appropriate chemotherapy.