Intravascular large B-cell lymphoma
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2] Syed Musadiq Ali M.B.B.S.[3]
Synonyms and keywords: Intravascular lymphomatosis, angiotropic large cell lymphoma, angio-endotheliotropic (intravascular) lymphoma, angioendotheliomatosis proliferans systematisata, malignant angioendotheliomatosis
Overview
Intravascular large B-cell lymphoma is a very rare subtype of diffuse large B-cell lymphoma (DLBCL). It is also considered a distinct type of non-Hodgkin lymphoma (NHL) in the World Health Organization (WHO) classification system. Intravascular large B-cell lymphoma affects small blood vessels. It is a rare and aggressive variant of intravascular proliferation of clonal lymphocytes with little to no parenchymal involvement. Based on the clinical presentation, intravascular large B-cell lymphoma may be classified into either Western variant or Asian variant. On microscopic histopathological analysis, diffuse infiltrate of large atypical cells with irregular nuclear contours, vesicular chromatin, and occasional prominent nucleoli are characteristic findings of intravascular large b-cell lymphoma. The incidence of intravascular large B-cell lymphoma increases with age; the median age at diagnosis is 67 years. Intravascular large B-cell lymphoma affects men and women equally. People with this type of lymphoma often have a poor prognosis. Symptoms of the intravascular large B-cell lymphoma include fever, weight loss, night sweats, chest pain, abdominal pain, bone pain, and painless swellings in the neck, axilla, groin, thorax, and abdomen. Lymph node biopsy is diagnostic of intravascular large B-cell lymphoma. CT, MRI, and PET scan may be helpful in the diagnosis of intravascular large B-cell lymphoma. The predominant therapy for intravascular large B-cell lymphoma is chemotherapy. Adjunctive radiotherapy may be required.
Classification
Based on the clinical presentation, intravascular large B-cell lymphoma may be classified into either Western variant or Asian variant.
| Name | Description |
|---|---|
| Western variant |
|
| Asian variant |
|
Pathophysiology
Intravascular large B-cell lymphoma is characterized by a massive intravascular proliferation of atypical mononuclear cells which lodged in the lumina of small or intermediate vessels in many organs. The neoplastic lymphoid cells are large with prominent nucleoli and frequent mitotic figures. Fibrin thrombi, haemorrhage and necrosis may be seen[1].
Microscopic Pathology
On microscopic histopathological analysis, diffuse infiltrate of large atypical cells with irregular nuclear contours, vesicular chromatin, and occasional prominent nucleoli are characteristic findings of intravascular large b-cell lymphoma.
Causes
There are no established causes for intravascular large B-cell lymphoma.
Differentiating type page name here from other Diseases
Intravascular large B-cell lymphoma must be differentiated from other diseases such as:
- Hepatosplenic T-cell lymphoma
- B-cell chronic lymphocytic leukemia
- Mantle cell lymphoma
- Splenic marginal zone lymphoma
Epidemiology and Demographics
Age
The incidence of intravascular large B-cell lymphoma increases with age; the median age at diagnosis is 67 years.[2]
Gender
Intravascular large B-cell lymphoma affects men and women equally.[2]
Risk Factors
There are no established risk factors for intravascular large B-cell lymphoma.
Natural History, Complications and Prognosis
- Intravascular large B-cell lymphoma usually affects the small blood vessels in many organs, including: central nervous system, kidneys, lungs, and skin. However, almost any site can be affected.
- People with intravascular large B-cell lymphoma have a variety of symptoms. The symptoms will depend on the tissue or organ affected when the blood vessels become occluded. This type of lymphoma is often difficult to diagnose because the symptoms can be so varied.
- Intravascular large B-cell lymphoma is a fast-growing (aggressive) lymphoma.
- People with this type of lymphoma often have a poor prognosis.
- People with this type of lymphoma can also develop a very serious condition where certain parts of the immune system are activated (hemophagocytic syndrome).[2]
- Hemophagocytic syndrome causes:
- Fever
- Enlarged liver and spleen (hepatosplenomegaly)
- A lower number of red blood cells, white blood cells and platelets in the blood (pancytopenia)
Diagnosis
Staging
Staging for Intravascular large B-cell lymphoma is provided in the following table:[3]
| Stage | Involvement | Extranodal (E) status |
|---|---|---|
| Limited | ||
| Stage I | One node or a group of adjacent nodes | Single extranodal lesions without nodal involvement |
| Stage II | Two or more nodal groups on the same side of the diaphragm | Stage I or II by nodal extent with limited contiguous extranodal involvement |
| Stage II bulky | II as above with "bulky" disease | Not applicable |
| Advanced | ||
| Stage III | Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement | Not applicable |
| Stage IV | Additional noncontiguous extralymphatic involvement | Not applicable |
Symptoms
People with intravascular large B-cell lymphoma have a variety of symptoms. The symptoms will depend on the tissue or organ affected when the blood vessels become occluded. This type of lymphoma is often difficult to diagnose because the symptoms can be so varied. Most common symptoms of the intravascular large B-cell lymphoma include:[2]
- Fever
- Weight loss
- Night sweats
- Painless swellings in the neck, axilla, groin, thorax, and abdomen
- Pain in the chest, abdomen, or bones
Physical Examination
Vitals
- Fever is often present
Skin
- Skin rash
HEENT
Thorax
- Thoracic masses suggestive of central lymphadenopathy
Abdomen
- Abdominal masses suggestive of central lymphadenopathy
- Hepatosplenomegaly
Extremities
| Organ Involved | Clinical features |
|---|---|
| Central nervous system |
|
| Cutaneous Involvement |
Laboratory Findings
Laboratory tests for intravascular large B-cell lymphoma include:[2]
- Complete blood count (CBC): pancytopenia
- Blood chemistry studies:
- Cytogenetic analysis
- Flow cytometry
- Immunohistochemistry
- Immunophenotyping
Biopsy
Lymph node biopsy is diagnostic of intravascular large B-cell lymphoma.
Other Imaging Findings
CT, MRI, and PET scan may be helpful in the diagnosis of intravascular large B-cell lymphoma.
Treatment
Medical Therapy
| Therapy[7] | Description |
|---|---|
| Chemotherapy |
|
| Radiation therapy |
|
References
- ↑ di Fonzo H, Contardo D, Carrozza D, Finocchietto P, Rojano Crisson A, Cabral C, de Los Angeles Juarez M (May 2017). "Intravascular Large B Cell Lymphoma Presenting as Fever of Unknown Origin and Diagnosed by Random Skin Biopsies: A Case Report and Literature Review". Am J Case Rep. 18: 482–486. doi:10.12659/ajcr.903816. PMC 5421743. PMID 28461685.
- ↑ 2.0 2.1 2.2 2.3 2.4 Intravascular large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf5ae3e27c3994bd54a7/. Accessed on March 09, 2016
- ↑ Cheson, Bruce D.; Fisher, Richard I.; Barrington, Sally F.; Cavalli, Franco; Schwartz, Lawrence H.; Zucca, Emanuele; Lister, T. Andrew; Alliance, Australasian Leukaemia and Lymphoma Group; Eastern Cooperative Oncology Group; European Mantle Cell Lymphoma Consortium; Italian Lymphoma Foundation; European Organisation for Research; Treatment of Cancer/Dutch Hemato-Oncology Group; Grupo Español de Médula Ósea; German High-Grade Lymphoma Study Group; German Hodgkin's Study Group; Japanese Lymphorra Study Group; Lymphoma Study Association; NCIC Clinical Trials Group; Nordic Lymphoma Study Group; Southwest Oncology Group; United Kingdom National Cancer Research Institute (2014-09-20). "Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification". Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology. 32 (27): 3059–3068. doi:10.1200/JCO.2013.54.8800. ISSN 1527-7755. PMID 25113753.
- ↑ Brett FM, Chen D, Loftus T, Langan Y, Looby S, Hutchinson S (May 2018). "Intravascular large B-cell lymphoma presenting clinically as rapidly progressive dementia". Ir J Med Sci. 187 (2): 319–322. doi:10.1007/s11845-017-1653-5. PMID 28726030.
- ↑ Kubisova K, Martanovic P, Sisovsky V, Tomleinova Z, Steno A, Janega P, Rychly B, Babal P (2016). "Dominant neurologic symptomatology in intravascular large B-cell lymphoma". Bratisl Lek Listy. 117 (6): 308–11. PMID 27546361.
- ↑ Intravascular large B-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/intravascular-large-b-cell-lymphoma/?region=nb. Accessed on March 9, 2016
- ↑ Shimada K, Kinoshita T, Naoe T, Nakamura S (September 2009). "Presentation and management of intravascular large B-cell lymphoma". Lancet Oncol. 10 (9): 895–902. doi:10.1016/S1470-2045(09)70140-8. PMID 19717091.
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