Symptoms And Signs Of Mantle Cell Lymphoma

  • Dr. Gugananda Prabu Bachelor of Dental Surgery - BDS, The Tamil Nadu Dr. M.G.R Medical University, Chennai, India
  • Alice Cui MSci Applied Medical Sciences, UCL
  • Arghavan KassraieBachelor of Engineering - BEng, Biomedical Engineering, University of Strathclyde

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Introduction

Mantle cell lymphoma (MCL) is a rare blood cancer type, form of type B cell Non-Hodgkin’s Lymphoma (NHL). When the white blood cells grow out of control and move around the lymphatic system, lymphoma develops.

In MCL, the cancerous changes begin in the mantle cell layer of the lymphoid follicles and these structures within lymph nodes play an important role in initiating immune responses. These B lymphocyte involvements are called B-cell non-Hodgkin’s lymphoma.

The classic symptoms of mantle cell lymphoma are known as B symptoms which are the symptoms of B-cell lymphoma such as night sweats, and unexplained weight loss, but can be without symptoms in its early stages. This article explores the signs and symptoms of mantle cell lymphoma, highlighting the importance of prompting individuals to seek medical attention.

How common is mantle cell lymphoma?

Mantle cell lymphoma (MCL) was identified as a separate non-Hodgkin lymphoma in 1992, since then it has been one of the most challenging forms of cancer to treat.1 MCL is often called an insidious disease because most of the time patients do not show any symptoms until it is more advanced. MCL comprises around 5 % of all non-Hodgkin's lymphomas, more common in men 3:1, and the median age at diagnosis ranges from 60 to 70 years old. The average incident rate in Europe is 0.5 to 1 case per 100,000 people.2

  • Age: Mostly prevalent in the elderly population, aged 65 and over
  • Family: A history of close family members can increase the risk3 
  • Previous cancer: Previous history of cancer and being treated with radiotherapy or chemotherapy could possibly develop non-Hodgkin’s lymphoma later in life.
  • Auto-immune diseases: Some of the diseases can be at risk of disease cancer
  • A weakened immune system
  • Infections: Some viruses or bacteria can also develop the risk of developing mantle cell lymphoma, but the probability remains small4

Mantle cell lymphoma and lymphadenopathy

Lymphadenopathy, the swelling of lymph nodes is a common sign of mantle cell lymphoma. In the human lymphatic system, the lymph nodes act as a filter that traps foreign substances and houses immune cells. In this cancer disease, the abnormal lymphocytes in this case white blood cells accumulate and cause enlargement. If the swollen lymph nodes do not go away after two weeks, it is one of the significant signs to see your doctor. 

Characteristics of MCL-related lymphadenopathy

  • Location: The most common sites are the neck, armpits, and groin, although any group of lymph nodes can be involved
  • Size: Swollen nodes can vary in size, painless and rubbery to palpation
  • Progression: Widespread at the time of diagnosis indicates the advanced stage of the disease

The presence of lymphadenopathy, especially if the lump on the body is hard, painless, and does not go away after two weeks, is a significant red flag for MCL. Further investigation by healthcare professionals to determine the underlying causes. Not all enlarged lymph nodes are indicative of cancer, some of it may be due to active infection or illness.5

Stage in disease spread

The patients at the initial stage do not show any symptoms, making it difficult to detect the disease early. Due to its ability to grow and spread quickly, mantle cell lymphoma is commonly detected in its later stages. Staging is important to understand disease spread.

  • Stage I: Cancer cells located in a single group of lymph nodes
  • Stage II: Cancer cells located in two separate lymph node groups/on one side of the diaphragm
  • Stage III: Cancer cells spread to both sides of the diaphragm and possibly to the spleen
  • Stage IV: Cancer cells spread outside the lymphatic system to other organs

B Symptoms and aggressive Lymphomas: Red flags for early detection

A group of systemic symptoms known as B symptoms play a crucial role in Mantle Cell lymphoma, although not specific to MCL but a strong indicator for further investigation. B symptoms are regularly associated with dysfunction of B lymphocytes, especially in MCL. The lymphocytes are responsible for several immune-related functions, especially in recognizing pathogens such as bacteria and viruses.

When cancer affects B cells, the early symptoms are

  • Unexplained fever: Mostly recurring without a clear cause like an infection
  • Night sweats: Excessive sweating at night not necessarily due to room temperature
  • Weight loss: Unusual weight loss of about 10% or more over a period of 6 months

An estimated 33 % of people living with mantle cell lymphoma are estimated to have B symptoms by the time they visit a doctor. 

Nonspecific symptoms

Nonspecific symptoms in people with mantle cell lymphoma include:

  • Appetite loss
  • Nausea and vomiting
  • Indigestion
  • Distended abdomen
  • Abdominal pain
  • Bloating
  • The sensation of “fullness”
  • Easy bruising
  • Fatigue
  • Lack of energy
  • Headaches
  • Increase in infections

Clinical signs

The clinical signs of mantle cell lymphoma can include,

  • Swollen lymph nodes
  • Enlargement of the spleen or liver
  • Unusual blood results and metabolic rates
  • Detection of cancerous cells under microscopic analysis
  • Absence of Reed-Sternberg cells
  • CD5, CD19, CD20, or CD22 antigens in immunohistochemistry testing
  • Translocations on the cyclin D1 (CCND1) gene

Less common symptoms of mantle cell lymphoma

There are also fewer common symptoms that can occur in some patients, and some are less frequent, being aware of them can be crucial for comprehensive diagnosis and early intervention. In some cases, this cancer disease affects the central nervous system which leads to symptoms like dizziness, headaches, numbness, tingling, or even seizures.6

Some patients may experience skin rashes, bumps, or lesions. It can sometimes trigger autoimmune responses, like autoimmune hemolytic anemia or thrombocytopenia. In some advanced cases, enlarged lymph nodes compress the superior vena cava. Bone marrow issues can also arise including cytopenias or increased susceptibility to bone fractures due to the complications.7

Importance of recognizing systemic symptoms and early detection

Early recognition of these symptoms and medical evaluation lead to a timely diagnosis and treatment initiation, which is crucial for optimal treatment outcomes. Symptoms can vary, some individuals may experience only mild symptoms or no B symptoms, while others may have more pronounced issues.

Improved treatment efficacy

Early-stage MCL treatment options, involve a combination of chemotherapy, immunotherapy, and potentially stem cell transplantation which increases the chances of achieving complete remission, where no detectable cancer remains. In the later stage of the disease, treatment options may be more limited, focusing on managing symptoms and controlling the disease rather than achieving complete remission.8

Reduced risk of complications

Early diagnosis allows us to begin the treatment before the disease's possible spread, minimizing the risk of complications like infections, bone damage, and organ dysfunction.

Enhanced quality of life

By managing common symptoms like fatigue, pain, and discomfort, the patients can maintain a more active and fulfilling life. 

Increased treatment options

A wider range of treatment possibilities includes clinical trials for promising new therapies that may not be available for patients with advanced disease. Tailoring therapy can be possible for the specific characteristics of the cancer.

Summary

It's important to remember that some of these symptoms can also be caused by various other conditions. It is crucial to consult a doctor for evaluation, particularly if any of these symptoms are encountered, further investigation is needed to rule out the disease or identify other potential causes. A thorough medical evaluation, including physical examination, blood investigation, imaging studies, and biopsy is essential for accurate diagnosis. Individuals can be more vigilant, potentially leading to a timely diagnosis and improved treatment outcomes.

References

  1. Smedby KE, Hjalgrim H. Epidemiology and etiology of mantle cell lymphoma and other non-Hodgkin lymphoma subtypes. Seminars in Cancer Biology [Internet]. 2011 [cited 2024 Jun 24]; 21(5):293–8. Available from: https://www.sciencedirect.com/science/article/pii/S1044579X11000599
  2. Lynch DT, Koya S, Acharya U, Kumar A. Mantle Cell Lymphoma. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 [cited 2024 Jun 24]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK536985/
  3. Wang SS, Slager SL, Brennan P, Holly EA, De Sanjose S, Bernstein L, et al. Family history of hematopoietic malignancies and risk of non-Hodgkin lymphoma (NHL): a pooled analysis of 10 211 cases and 11 905 controls from the International Lymphoma Epidemiology Consortium (InterLymph). Blood [Internet]. 2007 [cited 2024 Jun 24]; 109(8):3479–88. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1852242/
  4. Geisler CH, Kolstad A, Laurell A, Räty R, Jerkeman M, Eriksson M, et al. The Mantle Cell Lymphoma International Prognostic Index (MIPI) is superior to the International Prognostic Index (IPI) in predicting survival following intensive first-line immunochemotherapy and autologous stem cell transplantation (ASCT). Blood. 2010; 115(8):1530–3
  5. Argatoff LH, Connors JM, Klasa RJ, Horsman DE, Gascoyne RD. Mantle cell lymphoma: a clinicopathologic study of 80 cases. Blood. 1997; 89(6):2067–78
  6. Hoster E, Rosenwald A, Berger F, Bernd H-W, Hartmann S, Loddenkemper C, et al. Prognostic Value of Ki-67 Index, Cytology, and Growth Pattern in Mantle-Cell Lymphoma: Results From Randomized Trials of the European Mantle Cell Lymphoma Network. J Clin Oncol. 2016; 34(12):1386–94
  7. Romaguera JE, Medeiros LJ, Hagemeister FB, Fayad LE, Rodriguez MA, Pro B, et al. Frequency of gastrointestinal involvement and its clinical significance in mantle cell lymphoma. Cancer. 2003; 97(3):586–91
  8. Hitz F, Bargetzi M, Cogliatti S, Lohri A, Taverna C, Renner C, et al. Diagnosis and treatment of mantle cell lymphoma. Swiss Med Wkly. 2013; 143:w13868

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This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Dr Gugananda Prabu

Bachelor of Dental Surgery - BDS, The Tamil Nadu Dr. M.G.R Medical University, Chennai, India

Dr. Gugananda is a dental professional with extensive clinical experience, possessing a deep understanding of healthcare practices. His background extends beyond the clinic, encompassing valuable knowledge in healthcare research, clinical documentation, and review analysis in both the Indian and US healthcare systems. Dr. Gugananda is a passionate advocate for medical solutions and entrepreneurial ventures that transcend geographical boundaries. His expertise significantly contributes to advancing global healthcare paradigms.

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