1. Introduction
Intravascular large B-cell lymphoma(IVLBCL) is a rare form of large B-cell non-Hodgkin lymphoma characterized by selective proliferation of large cells within the lumen of small and medium-sized vessels of various organs.[1]IVLBCL shows a relatively high frequency ofCentral Nervous System(CNS), skin and bone marrow involvement.[2]IVLBCL limited to the CNS is rare, and the absence of extraneural features can delay diagnosis for quite some time.[3]Neurological symptoms were highly heterogeneous and included dementia, hemiparesis, seizures, myoclonus, and mental changes.[4]Diagnosis is challenging and is often made at autopsy or biopsy. Here we report a case of IVLBCL limited to the CNS presenting with acute and progressive dementia.attack, who was diagnosed by brain biopsy.
2 Case presentation
A previously healthy 47-year-old woman was brought to our hospital with a 6-month history ofrapidly progressive dementia(RPD), and weakness and numbness of the left extremity for 3 days. Six months earlier, he presented with a progressive decline in memory, decreased ability to calculate, and slow language speed. He often forgot to turn on the electricity and put out the fire when he was cooking. Orientation and visuoconstructive abilities were normal. There were no optical and acoustic hallucinations. There was no fever or weakness in her extremities. The mini-mental state examination (MMSE) score was 18. Vital signs, blood routines, liver and kidney function, electrolytes, lactate dehydrogenase (LDH), coagulation, tumor markers, and rheumatologic examinations were normal. . Magnetic resonance imaging (MRI) of the brain revealed multiple bilateral hyperintense lesions in the periventricular white matter, centro semiovale, corpus callosum, and cerebellum on T2 fluid-attenuated inversion recovery images (Fig. 1A1,A2), while the periventricular white matter was slightly enhanced on T1. enhanced images (Fig. 1A3) and punctate hyperintense lesions on diffusion-weighted images (Fig. 1A4). MRI of the spinal cord did not show any abnormalities. The electroencephalogram showed a mild abnormality. Lumbar puncture revealed an elevated cerebrospinal fluid (CSF) leukocyte count (20 × 106/L) and protein (0.89 g/L) with glucose and chloride in normal CSF. There were no malignant cells in the CSF cytology. Antibodies against nerve cell surface antigens and intracellular antigens were negative in serum and CSF. Myelin oligodendrocyte glycoprotein, glial fibrillary acidic protein, and antibodies against aquaporin-4 were not detected in serum, whereas the oligoclonal band result was normal. Deoxyribonucleic acids of common viruses were not detected in the CSF, such as herpes simplex virus, varicella-zoster virus, cytomegalovirus, Epstein-Barr virus. Idiopathic inflammatory demyelinating diseases were considered the most likely diagnosis. She was treated with intravenous methylprednisolone (1000 mg/d for 5 days, 500 mg/d for 3 days, 250 mg/d for 2 days, and 125 mg/d for 1 day), with subsequent oral methylprednisolone (20 mg/d) for 1 month and azathioprine (100 mg/d) as maintenance. Her clinical status partially improved with an MMSE score of 22 at 15 days after hospital discharge. Repeat brain MRI showed that the lesions decreased significantly at 2 months after discharge (Fig. 1 B1, B2, B3).
The patient suffered weakness and sudden numbness of the left limb and cognitive impairment 3 days before admission. She was treated with acuteattackat the local community hospital. And then, she was transferred to our hospital. On admission, he had impaired memory, impaired computational ability, impaired visuoconstructive ability, and disorientation with a score of 9 on the MMSE. Neurological examination revealed left facial palsy, dysarthria, left limb weakness (2/5 of muscle strength), and numbness with a positive Babinski. sign. Her LDH was elevated (338 U/L). The CSF revealed an elevated leukocyte count (10 × 106/L) and protein (1.77 g/L) with normal glucose and chlorine. CSF samples were negative for malignant cells. After 2 days of admission, he developed frequent generalized tonic-clonic seizures. Her condition deteriorated resulting in a coma after 4 days of admission. Brain MRI showed that lesions were enlarged and magnified on T2 fluid-attenuated inversion recovery and diffusion-weighted images (Fig. 1 C2, C3, C5), and some lesions had open ring enhancement on images enhanced T1 (Fig. 1 C4). Although the cerebral magnetic resonance angiography (MRA) was normal (Fig. 1 C1), cerebral vasculitis was considered due to the new symptoms ofattackand seizures. Brain tumor was not excluded as mass-like lesions on MRI. A brain biopsy of the right occipital lobe lesion was performed. The pathological specimen showed occlusion of the small vessels by neoplastic cells with prominent nucleoli (Fig. 2A). On immunostaining, these cells were found to be strongly positive for CD20 (Fig. 2B). No neoplastic cells were found in the bone marrow aspiration and in the random incisional skin biopsy. CT scans of the chest, abdomen, and pelvis were normal with no evidence of disease outside the CNS. The patient was diagnosed with IVLBCL limited to the CNS, but died of brain herniation after 10 days of admission.
3 Discussion and conclusions
The present patient's initial symptom was RPD. RPD is a syndrome caused by numerous pathological entities. Although RPD is not a rare manifestation in patients referred to neurological units, correct identification of the cause of RPD is a diagnostic challenge.[5]Previous observations from prion disease reference centers reported that sporadic Creutzfeldt-Jakob disease was the most common cause of RPD, followed by Alzheimer's disease.[6,7]Furthermore, 2 recent studies from India and Brazil reported that immune-mediated or infectious diseases of the CNS were the most common causes of RPD.[8,9]Our patient did not present visual hallucinations, myoclonus, dystaxia, or extrapyramidal signs. Brain MRI patterns did not show strictly cortical hyperintensities (“ribboning”) and hyperintensities in the basal ganglia. Therefore, the diagnosis of Creutzfeldt-Jakob disease was not established in the patient. Due to multiple lesions on brain MRI, neurodegenerative diseases, such as Alzheimer's disease, Lewy body dementia, Parkinson's dementia, were excluded. Antibodies against nerve cell surface antigens and intracellular antigens were negative. CSF virus results were normal. Evidence supporting autoimmune encephalitis or infectious diseases was insufficient. Oligoclonal band, myelin oligodendrocyte glycoprotein, glial fibrillary acidic protein, and aquaporin-4 antibodies were negative. However, the diagnosis of idiopathic inflammatory demyelinating diseases was considered, based on the spread of CNS lesions in space and time. The patient was treated with steroids with partial clinical and brain magnetic resonance improvement. Six months later, he developed acute left hemiparesis. Sharpattackwas initially considered at the community hospital. However, his cognitive function worsened. Repeated MRI of the brain showed that the lesions were obviously enlarged and increased. Cerebral vasculitis and tumor were raised. The patient was diagnosed with IVLBCL after a brain biopsy.
IVLBCL is a rare and fatal type of extranodal B-cell lymphoma, characterized by the proliferation of neoplastic cells in the lumen of small vessels, especially capillaries.[1,10]Two variants are described with cases in Western countries manifesting a relatively high frequency of rashes and multiple neurological deficits.[11]Those described in Asian countries preferably show a typical clinical hemophagocytic syndrome, represented by bone marrow involvement, fever, hepatosplenomegaly, and thrombocytopenia.[4]However, IVLBCL often presents with a set of nonspecific clinical features as a result of single or multiple organ involvement. Although any organ can be affected individually or in combination, the CNS and the skin are the most frequently affected organs.[1,12]During the clinical course of the disease, more than 60% of patients with IVLBCL will develop neurological symptoms, including encephalopathy,attack, seizures, RPD, myelopathy, radiculopathy, and neuropathy.[1,13]However, IVLBCL limited to the CNS is an extremely rare condition as IVLBCL is frequently found in multiple organs.[4]The most common laboratory test results for IVLBCL are anemia, elevated erythrocyte sedimentation rate, and elevated LDH.[14]Although various patterns of abnormal features on brain MRI have been reported in patients with IVLBCL, a recent study suggested that brain MRI findings were classified into 4 patterns: (1) non-specific white matter lesions, (2) lesions similar to heart attacks, (2) hyperintense lesions in the pons, and (4) thickening and/or enhancement of the meninges.[15]In contrast, MRA of the brain is not useful in the diagnosis of IVLBCL as the affected cerebral vessels are too small to show up directly on MRA.[sixteen]Given the characteristic that lymphoma cells in IVLBCL remain intravascular, PET is usually negative.[2]The first treatment option for these patients is the use of multi-agent anthracycline-based chemotherapy together with rituximab.[4]However, the prognosis of IVLBCL is poor with a high mortality rate. Our patient presented primarily with RPD and acuteattack, with elevated LDH and multiple hyperintense lesions on brain MRI. Although she was diagnosed with IVLBCL limited to the CNS by brain biopsy, she passed away due to rapid clinical aggravation.
We note several important lessons learned in this case. First, the accurate diagnosis of IVLBCL limited to the CNS is challenging and still depends mainly on histopathological examination due to the lack of specific clinical manifestations, laboratory markers and radiological features. Second, IVLBCL should be considered, when patients present with RPD andattackwith elevated LDH. Third, because appropriate treatment can improve outcomes, timely and correct diagnosis is essential for patients with IVLBCL.
author contributions
MW contributed to data analysis and interpretation and writing of the manuscript. YL and XH contributed to data collection and analysis. BZ contributed to the data interpretation of the manuscript and critical review of important intellectual content. All authors have read and approved the manuscript.
Conceptualization:Ming Wu.
Formal analysis:Yinyao Lin.
Investigation:Xuehonghuang.
Supervision:Bingjun Zhang.
Redacción – original blotter:Ming Wu.
Writing – proofreading and editing:Bingjun Zhang.
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Keywords:
case report;Central Nervous System;intravascular large B-cell lymphoma;rapidly progressive dementia;attack
FAQs
What chemotherapy drugs are given for large B-cell lymphoma? ›
The most common chemotherapy regimens for advanced DLBCL are: R-CHOP includes rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. The first four drugs are given into a vein (IV) over the course of one day, while prednisone is taken by mouth for five days.
What is the most useful drug that would target B-cell lymphoma? ›Rituximab (Rituxan).
Rituximab is a targeted therapy used to treat many different types of B-cell NHL. It works by targeting a molecule called CD20 that is located on the surface of normal B cells and B-cell NHL.
On April 19, 2023, the FDA approved Polivy® (polatuzumab vedotin-piiq) in combination with Rituxan® (rituximab), cyclophosphamide, doxorubicin and prednisone (R-CHP) for the treatment of adult patients who have previously untreated diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) or high-grade B- ...
What drugs treat diffuse B-cell lymphoma? ›The most widely used treatment for DLBCL presently is the combination known as R-CHOP (rituximab [Rituxan], cyclophosphamide [Cytoxan], doxorubicin [Adriamycin], vincristine [Oncovin], and prednisone) The R-CHOP regimen is usually given in 21-day cycles (once every 21 days) for an average of 6 cycles.
What is the second line treatment for large B-cell lymphoma? ›Occasionally, a patient will undergo an allogeneic transplant (patient receives stem cells from a donor). For those relapsed/refractory patients combination chemotherapy regimens are available. These second-line regimens include: ifosfamide, carboplatin, and etoposide (ICE)
What is the best treatment for B-cell lymphoma? ›The standard treatment for B-cell lymphoma is chemotherapy. If the disease is located in one area of the body (localized), the treatment plan may include surgery and a short, less intensive course of chemotherapy. If the disease is more advanced, the treatment usually includes high-dose chemotherapy.
Which B-cell lymphoma is aggressive? ›Less common forms of B-cell lymphoma include: Burkitt lymphoma: Considered the most aggressive form of lymphoma, this disease is one of the fastest growing of all cancers.
What is the cure rate for diffuse large B-cell lymphoma? ›What are survival rates for diffuse large B-cell lymphoma? According to the National Cancer Institute, 64.6% of people with DLBCL are alive five years after diagnosis. (Relative survival rates are estimates based on large groups of people.)
What chemo for lymphoma B? ›Chemotherapy is the main way to treat most types of B-cell lymphoma. You can get this on its own, or combine it with radiation or immunotherapy. Chemo uses drugs to kill fast-dividing cells in your body, including cancer cells. You get this medicine through a vein (IV), or you take it as a pill by mouth.
How is Stage 4 B-cell lymphoma treated? ›To treat stage 4 DLBCL, your doctor will likely recommend chemotherapy. For example, they may recommend the R-CHOP chemotherapy regimen. This involves a combination of the drugs cyclophosphamide, doxorubicin, vincristine, and prednisone, with the addition of rituximab, a monoclonal antibody.
What is the life expectancy of B-cell lymphoma? ›
Survival rates continue to improve as researchers identify more effective treatments. Approximately 65% of people diagnosed with the most common form of B-cell lymphoma are alive five years after diagnosis and considered cured.
What is the new FDA approved lymphoma treatment? ›FDA has approved Epkinly (epcoritamab-bysp) injection for the treatment of adult patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL), not otherwise specified, including DLBCL arising from indolent lymphoma, and high-grade B‑cell lymphoma after two or more lines of systemic therapy.
Is diffuse large B-cell lymphoma aggressive? ›Diffuse large B cell lymphoma (DLBCL) is the most common lymphoma, accounting for about 25% to 30% of all non-Hodgkin lymphoma. This disease presents as a rapidly growing mass or enlarging lymph nodes in a nodal or extranodal site. Though aggressive, it does respond well to chemotherapy.
How fast does diffuse B-cell lymphoma spread? ›They usually grow quite quickly, over just a few weeks. Sometimes, DLBCL can develop in lymph nodes deep inside your body where they can't be felt from the outside. The swollen nodes can form large lumps – known as 'bulky disease'. DLBCL can also develop outside lymph nodes, called 'extranodal' disease.
What is the immunotherapy for diffuse large B-cell lymphoma? ›Currently, axicabtagene ciloleucel (axi-cel, Yescarta®), tisagenlecleucel (tisa-cel, Kymriah®), and lisocabtagene maraleucel (liso-cel, Breyanzi®) have been approved by the US Food and Drug Administration (FDA) for the treatment of relapsed refractory DLBCL.
What is the prognosis of large B-cell lymphoma of the brain? ›Historically, the prognosis of primary central nervous system lymphoma has been very dismal, with overall survival of 1.5 months when untreated, and a 5-year survival rate of 30%.
Can Stage 4 large B-cell lymphoma be cured? ›However, nearly 70% of all patients with this subtype will have successful treatment. The earlier the cancer is detected and treated, the more likely it is that the disease will be curable.
What are the third line DLBCL treatments? ›Third-line therapy–CAR T-cells
CAR T-cells are curative for R/R DLBCL patients having either relapsed after ASCT or for transplant-ineligible patients. Thus, CAR T-cells represent the current standard of care for patients having received two previous lines of therapy.
Symptoms can start or get worse in just a few weeks. The most common symptom is one or more painless swellings. These swellings can grow very quickly.
What is the 10 year survival rate for diffuse large B cell lymphoma? ›Overall survival
OS according to IPI and R-IPI category is shown in Fig. 1; OS rates are shown in Table V. Among patients classified as having an IPI 'low risk', 80% were alive at 10 years; the 10-year OS rates in the 'low-intermediate', 'high-intermediate', and 'high-risk' groups were 60, 43, and 30%, respectively.
How do you treat aggressive lymphoma? ›
Chemotherapy is usually the main treatment. A combination of chemotherapy drugs is usually given along with targeted therapy. Radiation therapy is often given as well. If radiation therapy is going to be given after chemotherapy, fewer cycles of chemotherapy may be needed.
How bad is stage 4 B-cell lymphoma? ›Stage 4 lymphoma means that cancer has spread to an organ external to the lymphatic system. The survival rates vary widely depending on an individual's risk factors and type of cancer. The survival rate of stage 4 lymphoma is lower than that of the other stages, but doctors can cure the condition in some cases.
What are the signs of end stage lymphoma? ›- loss of appetite.
- fatigue and drowsiness.
- changes in breathing.
- confusion.
- withdrawal and loss of interest.
- feeling cold.
- loss of bladder and bowel control (incontinence)
- pain.
Richter's syndrome is a very rare type of high grade non-Hodgkin lymphoma (NHL). People who develop Richter's syndrome have a type of blood cancer called chronic lymphocytic leukaemia (CLL).
Can diffuse large B-cell lymphoma spread to the brain? ›Central nervous system diffuse large B-cell lymphoma (CNS-DLBCL), formerly named primary CNS lymphoma (PCNSL), is an extranodal aggressive lymphoma manifesting solely in the brain, spinal cord, leptomeninges or in the eye and represents the vast majority of CNS-lymphoma cases (1,2).
How treatable is large B-cell lymphoma? ›Diffuse large B-cell lymphoma can now be cured in more than 50% of patients.
What is Stage I diffuse large B-cell lymphoma? ›Stages of DLBCL
In DLBCL, there are four stages : Stage 1: The cancer affects only one area — either a single organ or a single cluster of lymph nodes. Stage 2: The cancer affects two or more areas on the same side of the diaphragm. Stage 3: The cancer affects areas on both sides of the diaphragm.
Background: Six cycles of R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) are the standard treatment for aggressive B-cell non-Hodgkin lymphoma.
What happens if you don't treat B-cell lymphoma? ›You may wonder about the impact on your long-term health if you delay treatment. But studies show that for people with slow-growing types of B-cell lymphoma, there is no difference in the way the disease develops between immediate treatment and watchful waiting.
Are there stages to B-cell lymphoma? ›Doctors divide DLBCL into four stages. Here's what they mean: Stage I: The cancer is in one lymph node area, lymph structure, or other site.
Can you survive large B-cell lymphoma? ›
Diffuse large B cell lymphoma (DLBCL) is the most common type of high grade lymphoma. Generally for people with DLBCL: 60 in 100 people (60%) will survive 5 years or more after their diagnosis.
At what stage is lymphoma terminal? ›Stage 4. Stage 4 is the most advanced stage of lymphoma. Lymphoma that has started in the lymph nodes and spread to at least one body organ outside the lymphatic system (for example, the lungs, liver, bone marrow or solid bones) is advanced lymphoma.
What is the cause of death of DLBCL patients? ›The causes of death were lymphoma relapse in 19%, cardiovascular in 23%, and other malignancies in 25% of the patients.
What is the immunotherapy pill for lymphoma? ›Rituximab (Rituxan) is the monoclonal antibody that doctors most often use to treat lymphoma. This drug targets the CD20 antigen, which many types of lymphoma make too much of. CD20 is found on a type of white blood cell called a B cell.
What are promising treatments for lymphoma? ›Immune checkpoint inhibitors that help T cells to better kill cancer cells have been effective in some people with recurrent Hodgkin lymphoma. Two such drugs—nivolumab (Opdivo) and pembrolizumab (Keytruda)—have been approved for some patients with Hodgkin lymphoma that has recurred after previous treatments.
Is Keytruda approved for lymphoma? ›FDA Approves Merck's KEYTRUDA® (pembrolizumab) for Adult and Pediatric Patients with Classical Hodgkin Lymphoma (cHL) Refractory to Treatment, or Who Have Relapsed After Three or More Prior Lines of Therapy.
How long can you live with stage 4 B-cell lymphoma? ›Stage 4 non-Hodgkin's diffuse large B-cell lymphoma has a five-year relative survival rate of 57%15. Stage 4 non-Hodgkin's follicular lymphoma has a five-year relative survival rate of 86%15. Stage 4 Hodgkin's lymphoma has a five-year relative survival rate of 82%16.
What virus causes diffuse large B-cell lymphoma? ›Infections: Some viral infections, including the human immunodeficiency virus (HIV), the human T-cell leukaemia/lymphoma virus (HTLV-1) and the Epstein-Barr virus (EBV) can increase the risk of someone developing DLBCL and other types of NHL.
What is 5 year survival for diffuse large B-cell lymphoma? ›Approximately 74% of patients with a localized stage of DLBCL survive 5 years, whereas 73% of those with a regional stage and 57% of those with a distant stage survive 5 years.
What is the most common site of diffuse large B-cell lymphoma? ›The majority of cases occur in lymph nodes with 40% in extranodal sites(4). These cases occur most frequently in the gastrointestinal tract, but may appear in any organ, including the skin, central nervous system (CNS), bone marrow (BM), salivary gland, lung, kidney, and liver(5,6).
Can B-cell lymphoma be caused by Covid? ›
Conclusion: We describe an uncommon case of COVID-19 who was finally diagnosed with B-cell lymphoma. An awareness of persistent fever and declined routine blood tests caused by hematological malignancies instead of COVID-19 itself can aid in providing appropriate guidelines for management and treatment.
How often does diffuse large B-cell lymphoma relapse? ›Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma. Approximately 30% to 40% of patients will develop relapsed/refractory (R/R) DLBCL, leading to significant morbidity and mortality.
Is diffuse large B-cell lymphoma painful? ›The main symptom of DLBCL is having swollen glands (lymph nodes). These won't necessarily be painful. The most common place for you to notice these would be in your neck, armpit or groin (at the top of your leg on the inside, where it meets your body).
What is the treatment for large cell lymphoma? ›DLBCL is usually treated with a number of different chemotherapy drugs, a steroid and a targeted immunotherapy drug called rituximab (Mabthera). You might hear this combination called chemoimmunotherapy. There are different combinations. One of the main combinations is R-CHOP.
Which B-cell lymphoma is most aggressive? ›Less common forms of B-cell lymphoma include: Burkitt lymphoma: Considered the most aggressive form of lymphoma, this disease is one of the fastest growing of all cancers.
How aggressive is B-cell lymphoma? ›More than 18,000 people are diagnosed with DLBCL each year. Diffuse large B-cell lymphoma is an aggressive (fast-growing) NHL that affects B-lymphocytes. Lymphocytes are one type of white blood cell. B-cells are lymphocytes that make antibodies to fight infections and are an important part of the lymphatic system.
How do you treat Stage 4 B-cell lymphoma? ›To treat stage 4 DLBCL, your doctor will likely recommend chemotherapy. For example, they may recommend the R-CHOP chemotherapy regimen. This involves a combination of the drugs cyclophosphamide, doxorubicin, vincristine, and prednisone, with the addition of rituximab, a monoclonal antibody.
How fast does large B-cell lymphoma spread? ›They usually grow quite quickly, over just a few weeks. Sometimes, DLBCL can develop in lymph nodes deep inside your body where they can't be felt from the outside. The swollen nodes can form large lumps – known as 'bulky disease'. DLBCL can also develop outside lymph nodes, called 'extranodal' disease.
What is the 10 year survival rate for diffuse large B-cell lymphoma? ›Overall survival
OS according to IPI and R-IPI category is shown in Fig. 1; OS rates are shown in Table V. Among patients classified as having an IPI 'low risk', 80% were alive at 10 years; the 10-year OS rates in the 'low-intermediate', 'high-intermediate', and 'high-risk' groups were 60, 43, and 30%, respectively.
Blastic NK cell lymphoma
This very rare type of T cell lymphoma only affects a few people each year. It usually affects adults. Blastic NK cell lymphoma tends to grow very quickly and can be difficult to treat. It can start almost anywhere in the body.
What are the new treatments for diffuse large cell lymphoma? ›
May 19, 2023 – The U.S. Food and Drug Administration (FDA) announced it has approved epcoritamab-bysp (Epkinly, co-developed by Genmab and AbbVie) for relapsed or refractory diffuse large B-cell lymphoma (DLBCL) after two or more lines of systemic therapy. More information.
What is aggressive large cell lymphoma? ›Large cell lymphoma is an aggressive form of NHL, which is a type of blood cancer that begins in the lymphatic system. The two main types of large cell lymphoma are DLBCL and ALCL. These aggressive blood cancers respond well to treatment, especially if doctors detect and treat them early.
What is the life expectancy of someone with diffuse large B-cell lymphoma? ›DLBCL is an aggressive blood cancer that can be rapidly fatal if left untreated. Without medical attention, the average lifespan of a patient with a diagnosis of DLBCL is less than 1 year.
What are the stages of large B cell lymphoma? ›Stage 1: The cancer affects only one area — either a single organ or a single cluster of lymph nodes. Stage 2: The cancer affects two or more areas on the same side of the diaphragm. Stage 3: The cancer affects areas on both sides of the diaphragm.