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 Table of Contents  
Year : 2022  |  Volume : 47  |  Issue : 3  |  Page : 222-223

A rare case report of Intravascular Diffuse Large B cell Lymphoma presenting as subcutaneous nodules

1 Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
2 Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

Date of Submission22-Feb-2021
Date of Acceptance16-Mar-2021
Date of Web Publication03-Jan-2023

Correspondence Address:
Gopan Gayatri
Senior Resident in Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ejh.ejh_15_21

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Intravascular diffuse large B-cell lymphoma is a rare entity where the large lymphoma cells are seen within the vessels. The difficulty is in its diagnosis, as it can have varied presentations affecting multiple systems. Here, we present the case report of a woman with intravascular diffuse large B-cell lymphoma who presented with subcutaneous nodules.

Keywords: cutaneous lymphoma, intravascular diffuse large B-cell lymphoma, subcutaneous nodules

How to cite this article:
N P P, Gayatri G, Vishnu H, A V J. A rare case report of Intravascular Diffuse Large B cell Lymphoma presenting as subcutaneous nodules. Egypt J Haematol 2022;47:222-3

How to cite this URL:
N P P, Gayatri G, Vishnu H, A V J. A rare case report of Intravascular Diffuse Large B cell Lymphoma presenting as subcutaneous nodules. Egypt J Haematol [serial online] 2022 [cited 2023 Mar 30];47:222-3. Available from: http://www.ehj.eg.net/text.asp?2022/47/3/222/366860

  Case report Top

A 56-year-old woman with no previous comorbidities was referred to our institute for the evaluation of multiple subcutaneous swellings, to rule out the possibility of cutaneous lymphoma. She complained of pain in her lower limbs along with multiple swellings of 1-month duration. There was no history of fever, weight loss, ornight sweats. There was no pruritus or rash. On examination, there were multiple tender erythematous nodules about 2 × 2 cm over both her legs extending up to thigh. No nodules could be found elsewhere. All othersystems were within normal limits. FNAC from the swelling was suggestive of lymphoma. We proceeded with the investigations. Her blood counts and biochemical parameters were within normal limits. Peripheral smear and bone marrow studies were within normal limits. Whole-body computed tomography finding was normal except for small volume cervical lymphadenopathy.

Biopsy of the subcutaneous swelling was suggestive of intravascular diffuse large B-cell lymphoma (IVDLBCL). PAX5, CD 20, BCL6, and MUM 1 were positive, and CD 10 and CD 5 were negative. MIB 1 was 90%. She was treated with six cycles of R CHOP (Rituximab, cyclophosphamide, doxorubicin, vincristine, and steroids) ([Figure 1] and [Figure 2]).
Figure 1: Subcutaneous nodules in the thigh.

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Figure 2: Large B cells within the lumen of blood vessels.

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  Discussion Top

IVDLBCL is a rare lymphoma entity in which large neoplastic cells grow within the lumen of blood vessels [1].

Why IVDLBCL cells remain within the lumen is intriguing. There have been some studies that throw light in this area [2]. They show that these DLBCL cells express molecules necessary for migration and adhesion to endothelium but lack cells involved in extravasation. This makes them different from DLBCL. They lack CD29 which is critical for extravasation. IVLBCL cells express Cxc3 and Cxcr4 (involved in lymphocyte trafficking and integrin activation) but lack the respective ligands. IVLBCL does not seem to express matrix metalloproteinase-2 and −9, the two molecules important for parenchymal invasion [3].

There are different types of IVDLBCL.

  • (1) Cohesive, where neoplastic cells are seen attached to the walls and filling the lumen thus making the study of architecture of vessels very difficult.

  • (2) Discohesive, where the cells are seen in the center of the lumen in a free-floating manner.

  • (3) Marginal, in which cells are attached to the endothelium leaving the center of the lumen free.

Although DLBCL cells are seen in the lumen, peripheral smear examination does not show evidence of these cells.

There are mainly three clinical types [4]. Classic variant is associated with fever and multiple organ dysfunction. Skin involvement can occur in the form of plaques, papules, erythematous areas, tumors, and ulcerated nodules [5]. Cutaneous variant is different from the classic variant in that isolated skin involvement can occur and is associated with a good prognosis and longer median survival. Hemophagocytic variant is associated with fever, hepatosplenomegaly, and thrombocytopenia. It has a devastating course and has survival measured in months [6].

Stage IE is seen in 40% of cases. The remaining 60% of patients with IVLBCL almost invariably show stage IV disease. Staging workup should include routine magnetic resonance imaging of the CNS coupled with bone marrow biopsy, which plays the dual role of both a diagnostic and staging tool. The addition of rituximab to CHOP chemotherapy has revolutionized the treatment of IVDLBCL [7],[8],[9]. This may be owing to the high drug concentrations and high intravascular levels of complements. This may also cause severe infusion reactions, leading to treatment interruptions. So rituximab can be delayed and can be given on day 4 or 5 of chemotherapy. Upfront andsalvage options must include rituximab. Except for cutaneous IVDLBCL, all others have a highly aggressive course. The use of high-dose chemotherapy supported by autologous stem cell transplantation remains a matter of debate [10].

  Conclusion Top

IVDLBCL is an aggressive neoplasm with a poorprognosis which presents with multisystem involvement. Rituximab containing chemoimmunotherapy is the treatment of choice. Here we have presented a case report of IVDLBCL to bring into highlight its varied presentations and its aggressive nature.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Ponzoni M, Ferreri AJ, Campo E, et al. Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol 2007; 25:3168–3173.  Back to cited text no. 1
Takahashi E, Kajimoto K, Fukatsu T, Yoshida M, Eimoto T, Nakamura S Intravascular large T-cell lymphoma: a case report of CD30- positive and ALK-negative anaplastic type with cytotoxic molecule expression. Virchows Arch 2005; 447:1000–1006.  Back to cited text no. 2
Ponzoni M, Arrigoni G, Gould VE, Del Curto B, Maggioni M, Scapinello A, et al. Lack of CD 29 (beta1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis. Hum Pathol 2000; 31:220–226.  Back to cited text no. 3
Ferreri AJM, Dognini GP, Campo E, Willemze R, Seymour JF, Bairey O, et al. International Extranodal Lymphoma Study Group (IELSG). Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions. Haematologica 2007; 92:486–492.  Back to cited text no. 4
Matsue K, Hayama BY, Iwama K, Koyama T, Fujiwara H, Yamakura M, et al. High frequency of neurolymphomatosis as a relapse disease of intravascular large B-cell lymphoma. Cancer 2011; 117:4512–4521.  Back to cited text no. 5
Gill S, Melosky B, Haley L, ChanYan C Use of random skin biopsy to diagnose intravascular lymphoma presenting as fever of unknown origin. Am J Med 2003; 114:56–58.  Back to cited text no. 6
Shimada K, Matsue K, Yamamoto K, Murase T, Ichikawa N, Okamoto M, et al. Retrospective analysis of intravascular large B-cell lymphoma treated with rituximab containing chemotherapy as reported by the IVL study group in Japan. J Clin Oncol 2008; 26:3189–3195.  Back to cited text no. 7
Ferreri AJ, Dognini GP, Bairey O, Szomor A, Montalbán C, Horvath B, et al. International Extranodal Lymphoma Study Group. The addition of rituximab to anthracycline-based chemotherapy significantly improves outcome in ‘Western’ patients with intravascular large B-cell lymphoma. Br J Haematol 2008; 143:253–257.  Back to cited text no. 8
Ferreri AJ, Dognini GP, Govi S, Crocchiolo R, Bouzani M, Bollinger CR, et al. Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma?. JClin Oncol 2008; 26:5134–5136.  Back to cited text no. 9
Meissner J, Finel H, Dietrich S, Boumendil A, Kanfer E, Laboure G, et al. Autologous hematopoietic stem cell transplantation for intravascular large B-cell lymphoma: the European Society for Blood and Marrow Transplantation experience. Bone Marrow Transplant 2017; 52:650–652.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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