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

Prakash N P; Gopan Gayatri; Hari Vishnu; Jayasudha A V

doi:10.4103/ejh.ejh_15_21

CASE REPORT

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

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

Prakash N P¹, Gopan Gayatri¹, Hari Vishnu¹, Jayasudha A V²
¹ Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
² Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

Date of Submission	22-Feb-2021
Date of Acceptance	16-Mar-2021
Date of Web Publication	03-Jan-2023

Correspondence Address:
Gopan Gayatri
Senior Resident in Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ejh.ejh_15_21

Abstract

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

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

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

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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