Sézary disease, or Sézary syndrome, is a type of cutaneous T-cell lymphoma that was first described by

Albert Sézary Albert Sézary (26 December 1880, Algiers – 1 December 1956, Paris) was a French dermatologist and syphilogist. He served as a hospital interne in Algiers (from 1901) and Paris (from 1905), where he worked with neurologists Joseph Jules Dej ...

. The affected

T cells A T cell is a type of lymphocyte. T cells are one of the important white blood cells of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes by the presence of a T-cell re ...

, known as Sézary's cells or Lutzner cells, have pathological quantities of

mucopolysaccharide Glycosaminoglycans (GAGs) or mucopolysaccharides are long, linear polysaccharides consisting of repeating disaccharide units (i.e. two-sugar units). The repeating two-sugar unit consists of a uronic sugar and an amino sugar, except in the case o ...

s. Sézary disease is sometimes considered a late stage of

mycosis fungoides Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesion ...

with lymphadenopathy.

Signs and symptoms

Sézary disease and

are cutaneous T-cell lymphomas having a primary manifestation in the

skin Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different de ...

. The disease's origin is a peripheral CD4+ T-lymphocyte, although rarer CD8+/CD4- cases have been observed. Epidermotropism (lymphocytes residing in the epidermis) by

neoplastic A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

CD4+ In molecular biology, CD4 (cluster of differentiation 4) is a glycoprotein that serves as a co-receptor for the T-cell receptor (TCR). CD4 is found on the surface of immune cells such as T helper cells, monocytes, macrophages, and dendritic ...

lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. Although the condition can affect people of all ages, it is commonly diagnosed in adults over age 60. The dominant signs and symptoms of the disease are: # Generalized

erythroderma Erythroderma is an inflammatory skin disease with redness and scaling that affects nearly the entire cutaneous surface.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. p. 436. . This term applie ...

– redness of the skin # Lymphadenopathy – swollen, enlarged lymph nodes # Atypical

– malignant lymphocytes known as "Sézary cells" seen in the peripheral blood with typical cerebriform nuclei (brain-shaped, convoluted nuclei) #

Hepatosplenomegaly Hepatosplenomegaly (commonly abbreviated HSM) is the simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly). Hepatosplenomegaly can occur as the result of acute viral hepatitis, infectious mononucleosis, and his ...

– enlarged

liver The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...

and

spleen The spleen is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes .

Palmoplantar keratoderma Palmoplantar keratodermas are a heterogeneous group of disorders characterized by abnormal thickening of the stratum corneum of the palms and soles. Autosomal recessive, dominant, X-linked, and acquired forms have all been described. Types C ...

– thickening of the palms of the hands, and soles of the feet

Diagnosis

Those who have Sézary disease often present with skin lesions that do not heal with normal medication. A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma. Finally, a

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a dise ...

of a skin lesion can be performed to rule out any other causes. The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences: # More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome # Sometimes absent epidermotropism # Increased lymph node involvement with infiltrates of Sézary syndrome.

Treatment

Treatment typically includes some combination of

photodynamic therapy Photodynamic therapy (PDT) is a form of phototherapy involving light and a photosensitizing chemical substance, used in conjunction with molecular oxygen to elicit cell death (phototoxicity). PDT is popularly used in treating acne. It is used cl ...

radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radi ...

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemothe ...

, and biologic therapy. Treatments are often used in combination with phototherapy and chemotherapy, though pure chemotherapy is rarely used today. No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.

Radiation therapy

A number of types of radiation therapy may be used including total skin electron therapy. While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin. It is only available at a few institutions.

Chemotherapy

Romidepsin Romidepsin, also known as Istodax, is an anticancer agent used in cutaneous T-cell lymphoma (CTCL) and other peripheral T-cell lymphomas (PTCLs). Romidepsin is a natural product obtained from the bacterium ''Chromobacterium violaceum'', and wo ...

vorinostat Vorinostat (rINN) also known as Suberoylanilide hydroxamic acid ( suberoyl+anilide+ hydroxamic acid abbreviated as SAHA) is a member of a larger class of compounds that inhibit histone deacetylases (HDAC). Histone deacetylase inhibitors (HDI) ...

and a few others are a second-line drug for cutaneous T-cell lymphoma.

Mogamulizumab Mogamulizumab, sold under the brand name Poteligeo, is a humanized, afucosylated monoclonal antibody targeting CC chemokine receptor 4 (CCR4). The U.S. Food and Drug Administration (FDA) approved it in August 2018 for treatment of relapsed or ...

has been approved in Japan and the United States.

Epidemiology

In the Western population, there are around 3 cases of Sézary syndrome per 1,000,000 people. Sézary disease is more common in males with a ratio of 2:1, and the mean age of diagnosis is between 55 and 60 years of age.

References

External links

Sezary Syndrome lymphoma information
{{DEFAULTSORT:Sezary's Disease Lymphoma Lymphoid-related cutaneous conditions Syndromes