What is Sezary Syndrome ?
Sezary Syndrome, or Sezary disease, is defined as a more aggressive variant of a form of cancer known as cutaneous T-cell lymphoma (CTCL) that belongs to a broad group of diseases known as non-hodgkin’s lymphomas.
Other than humans, the disorder has also been reported in cats and dogs.
Image Source via Wikimedia Commons
Sezary Syndrome History
It was described initially by the French syphilologist and dermatologist Albert Sézary. This disease is occasionally believed to be a later stage of the condition mycosis fungoides [2] accompanied by lymphadenopathy.
Sezary Syndrome Epidemiology
A very rare skin disorder, it occurs in only 3 out of 1000000 people. It is more frequently seen in males, and the ratio between affected men and women is 2:1. The average age during the time of diagnosis is around 55 to 60 years.
Sezary Syndrome Causes
The underlying factors are not currently known [3]. A peripheral CD4+ T-lymphocyte is suspected as the cause. In some rare cases, however, an involvement of CD8+/CD4- has also been observed. Most sufferers have one or more chromosomal anomalies, such as the gain or loss of a genetic material, which usually occur during the lifetime. These can be spotted only in the genetic material or DNA of the cancerous cells. Patients tend to have lost DNA material from the regions of chromosomes 17 and 10 or additions of DNA material to the regions of the chromosomes 17 and 8.
The inheritance pattern has not been fully determined yet. It can occur in people having no family history, and is hence not believed to be hereditary in most cases.
Sezary Syndrome Symptoms
The dominant signs include:
- Pruritus
- Alopecia
- Ectropion
- Facies leonine
- Onychodystrophy
- Hepatosplenomegaly
- Peripheral adenopathies
- Palmo-plantar keratoderma
- Changes in the hair, nails, or eyelids
- Generalized exfoliative erythroderma
- Reddish, peeling, itchy and painful skin
- Sensations of feeling cold and shivering
- Lymphadenopathy or enlarged lymph nodes
- Presence of tumors, patches or plaques on the skin
- Presence of atypical T-cells (i.e., Sezary cells) in peripheral blood
Symptoms like mycosis fungoides, are mainly evident on the skin. The distinctive characteristics include lesions (Pautrier’s microabscesses) and Epidermotropism by neoplastic CD4+ lymphocytes. Development of Sezary cells (cancerous T-cells) on skin, blood and lymph nodes are also noticed.
Sezary Syndrome Diagnosis
Diagnosis involves a thorough physical examination and evaluation of the medical history of patients. Regular medications are often unable to heal skin lesions. The following diagnostic tests may then be recommended:
- Immunogenotyping
- Flow cytometry tests
- Immunophenotyping
- Bone marrow biopsies
- Blood tests (Complete blood count with differential)
- Peripheral blood smear
A number of imaging tests might be required to see if the cancer has metastasized to lymph nodes or any other organs. These may include:
- CAT scans
- Magnetic resonance imaging (MRI) scans
- Positron emission tomography (PET) scans
Sezary Syndrome Differential Diagnosis
It includes isolating the symptoms from those of disorders like [4]:
- Psoriasis
- Parapsoriasis
- Atopic Eczema
- Actinic reticuloid
- Atopic dermatitis
- Contact dermatitis
- Scabies Norvegica
- Mycosis fungoides
- Diffuse Mastocytosis
- Ichthyosis Congenita
- Seborrheic Dermatitis
- Pemphigus Foliaceus
- Pityriasis rubra pilaris
- Psoriatic Erythroderma
- Idiopathic skin diseases
- Graft-versus-host disease
- Keratosis palmo-plantaris
- Chronic actinic dermatitis
- Cutaneous drug reactions
- Severe seborrheic dermatitis
- Hypereosinophilic syndrome
- Severe eczematous reactions
- Chronic lymphocytic leukemia
- T-cell prolymphocytic leukemia
- Adult T-cell leukemia lymphoma
- Drug-induced pseudolymphoma reactions
- Other hematologic or lympho-proliferative diseases
- Photosensitivity conditions such as lupus, dermatomyositis
Sezary Syndrome Treatment
The main aim of therapy is to destroy the malignant lymphocytes as well as correct the immune imbalances that occur due to the malignant Th2 lymphocytes.
Treatment options include:
- Immunotherapeutic drugs (retinoids, interferons, extracorporeal photopheresis) as well as combination therapy
- Vorinostat (Zolinza), classified as a second-line medication for cutaneous T-cell lymphomas.
- Combination of phototherapy and chemotherapy.
- Bone marrow transplantation and systemic chemotherapy.
- Chemotherapeutics, such as gemcitabine and doxorubicin and monoclonal antibodies like alemtuzumab
No single mode of therapy stands apart with clear-cut benefits when compared to other treatment modalities and management of all cases of SS are challenging.
Sezary Syndrome Prognosis
The prognosis depends on the stage the cancer is in as well as the type of lesions that are evident, such as plaques, patches, or tumors. It is difficult to cure this syndrome. Treatment is mostly palliative, aiming at relieving the symptoms and improving the overall quality of life. The estimated 5-year survival rate for this disease is around 20-27%.
Sezary Syndrome Pictures
The following images demonstrate the nature of the lesions observed in Sezary’s syndrome.
Picture 1 – Sezary Syndrome
Picture 2 – Sezary Syndrome Image
Sezary Syndrome ICD 9 code
The ICD 9 code for this disease is 202.2 [1].