Q:

9/30/2013
A 28 year old heterosexual white male, never smoked tobacco, no ETOH or illicits with no significant PMH presented with a 4 year Hx of truncal parapsoriasis, now with biopsy positive cutaneous t-cell lymphoma, folliculotropic mycosis fungoides, Stage 2B with malignant clones in the blood. Initial lab abnormalities prior to treatment included serum IgM 21 per immunofixation, all other immunoglobins WNL, Kappa Free Light Chains 53 reducing to normal in 3 months, Kappa/Lambda ratio high also reducing to normal in 3 months, 25-Hydroxy 20 supplemented with oral vitamin D. Extensive testing has excluded leukemia, multiple myeloma, HIV and other immune deficiencies. Further work-up and treatment has been limited by the loss of health insurance, low income primary care has been managing his care. Is there any strategy recommended to enhance immune function to assist in the control of his non-Hodgkin Lymphoma?

A:

Thank you for your inquiry.

Patients with mycosis fungoides are susceptible to infection because of immune dysfunction as well as physical factors. Although it is basically a tumor of T cells, they can have both impaired cellular and humoral immunity. They can experience both viral (e.g., herpes) and bacterial infections. They also can show cutaneous colonization with Staphylococcus aureus in the skin and nose. Such colonized patients often respond to treatment with antibiotics to eradicate the bacteria, showing signs of clinical improvement in skin involvement with the lymphoma. Patients with advanced disease frequently die of infections rather than tumor burden. Although there may be cases treated with intravenous immunoglobulin, I have not been able to find such with a search of the literature.

In summary, although infections are important in causing morbidity and fatality in patients with mycosis fungoides, except for the measures above, I have not been able to find any specific studies on other measures to prevent such infections.

Thank you again for your inquiry and we hope this response is helpful to you.

PubMed
Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome.
Krejsgaard T, Odum N, Geisler C, Wasik MA, Woetmann A
Leukemia. 2012 Mar;26(3):424-32. Epub 2011 Sep 09.
Cutaneous T-cell lymphoma (CTCL) is the term for diseases characterized by primary accumulation of malignant T cells in the skin. Patients with the two predominant clinical forms of CTCL called mycosis fungoides (MF) and Sézary syndrome (SS) characteristically develop severe immunodeficiency during disease progression and consequently patients with advanced disease frequently die of infections and not from the tumor burden. For decades, it has been suspected that the malignant T cells actively drive the evolving immunodeficiency to avoid antitumor immunity, yet, the underlying mechanisms remain unclear. The identification of a subset of highly immunosuppressive regulatory T cells (Tregs) triggered a variety of studies investigating if MF and SS are malignant proliferations of Tregs but seemingly discordant findings have been reported. Here, we review the literature to clarify the role of Tregs in MF and SS and discuss the potential mechanisms driving the immunodeficiency.

Prevalence and treatment of Staphylococcus aureus colonization in patients with mycosis fungoides and Sézary syndrome.
Talpur R, Bassett R, Duvic M
Br J Dermatol. 2008;159(1):105.
Background: Mycosis fungoides (MF) and Sézary syndrome (SS), variants of cutaneous T-cell lymphoma, may arise from antigen-driven clonal expansion and accumulation of helper-memory T cells. Superantigens from Staphylococcus aureus can stimulate T cells.
Objectives: (i) To determine the prevalence of S. aureus carriage in nares and skin in patients with MF/SS compared with historical rates in other conditions. (ii) To determine whether eradication of S. aureus carriage is associated with clinical improvement. Methods Skin and nares cultures were performed prospectively. Patients with positive nares and skin cultures were treated with oral antibiotics and intranasal mupirocin 2% and samples were taken for reculturing at 3 days, 4 weeks and 8 weeks. An exact binomial test was used to compare the carriage rates among different groups.
Results: Among 106 patients with MF/SS, 67 (63%) had skin colonization and 57 (54%) had nasal colonization. Staphylococcus aureus was isolated from 44 patients, 33 (31%) each from skin and nares. Colonization was highest in erythrodermicSS (48%), similar to atopic dermatitis (64%), and lowest in MF without erythroderma (26%), psoriasis (21%), and the general population (10%). Oral and topical antibiotics eradicated S. aureus colonization in nares in 28 of 33 (85%) patients and in MF skin lesions in 30 of 33 (91%) patients at 4-8 weeks, with rapid clinical improvement seen in 58% of S. aureus-colonized patients.
Conclusions: Staphylococcal carriage in nares and skin lesions of patients with MF is similar to that in atopic dermatitis. Eradication of staphylococci from the skin is possible with treatment and was associated with clinical improvement.

Sincerely,
Phil Lieberman, M.D.

AAAAI - American Academy of Allergy Asthma & Immunology