MANTLE CELL LYMPHOMA

Mantle Cell Lymphoma is a malignancy that originates from the mantle zone area of the lymph nodes than gives rise to poorly functioning B cell lymphocytes which are responsible for the immunity of an individual.  It is a very slow-growing tumor and could sometimes pass off as normal lymphocytes in a bone marrow smear to an untrained eye. It has a very unfavorable character in that this type of lymphoma has a high tendency to recur even if the patient is considered to be free of the condition.

Mantle cell lymphoma currently affects 6% of the worldwide population. Commonly found in the middle aged to older men than in women and is believed to be caused by a displacement of the loci between chromosome 11 and 14. Due to this “mix-up,” there is also aberrant cyclin D1 which is responsible for the regulating cell growth, thereby causing overproduction of B cells.

TYPES

Mantle Cell lymphoma is classified based on their degree of involvement:

Stage 1- Presence of malignant lymph nodes in once cluster or location

Stage II- Presence of two or more nodes occurring on the same side or location

Stage III- Two or more malignant lymph nodes located both on the upper and lower portions of the diaphragm

Stage IV- Presence of malignant lymph nodes scattered all over the body and non-contiguous sites of the primary lesion

SYMPTOMS

Patients usually seek to consult in the later stages of the disease because of the fast progression of the lymphoma. Their complaints may present as either of the following:

  • Low-grade fever that occurs during the evening
  • Losing weight more than 10 % of the usual body weight
  • Lumps and bumps on any part of the body (usually found under the chin, the armpits and the area in between the abdomen and the thigh)
  • Unusual reaction to insect bites (some patients report pus formation on the area of the bite while some experience the appearance of wheal that takes a long time to resolve).

DIAGNOSIS

  • Lymph node biopsy with the identification of surface markers for B cells
  • Immunohistochemical testing for the presence of overproduction of Cyclin D1 protein
  • Complete blood counts
  • Imaging scans of the whole body, computed tomography scans on the area where the nodes are palpated and other adjacent areas
  • Bone marrow aspiration and biopsy – to identify the presence of predominant cells
  • Quantitative testing for Lactate dehydrogenase and Beta 2 microglobulin levels
  • Detection of prognostic markers such as Ki-67 serves as a gauge for the replication potential of the malignant cells

TREATMENT

Options for treatment depends mostly on the behavior of the lymphoma: 

  • Slow growing lymphomas are closely monitored and observed. The patient is advised to adhere to their scheduled clinic visits, and diagnostic imaging tests are done regularly. Treatment is initiated when there is a note of the progression of the size of the node as compared to the baseline
  • Chemotherapy using the following drugs: Bortezomib, Rituxan, Cyclophosphamide, Adriamycin and prednisone.

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