Schnitzler Syndrome Specimens

Bay Biosciences provides high-quality, matched fresh frozen sera (serum),  plasma, and peripheral blood mononuclear cells (PBMC) bio-fluids from patients diagnosed with Schnitzler syndrome.

Moreover, the sera (serum), plasma and PBMC bio-fluids are processed from Schnitzler syndrome patient’s peripheral whole-blood using customized collection and processing protocols.

In addition, the matched bio-fluids are collected from unique patients with Schnitzler syndrome and are provided to a valued pharmaceutical customer for research, development and drug discovery.

Schnitzler Syndrome Overview

Schnitzler Syndrome also known as Chronic Urticaria is a rare autoinflammatory disease characterized by onset around middle age of chronic hives (Urticaria) and periodic Fever, arthritis (bone pain and joint pain), sometimes with joint inflammation, weight loss, malaise, fatigue, swollen lymph glands, hematological abnormalities, organomegaly (abnormally enlarged organs), enlarged spleen and liver. The exact underlying cause of the condition is unknown; however, most cases occur sporadically in patients with no family history of the condition. Treatment is focused on alleviating the signs and symptoms associated with the condition and may include various medications and/or phototherapy.

Schnitzler Syndrome is considered an autoinflammatory disorder and is generally treated with anakinra, which inhibits interleukin-1. This treatment controls the condition but does not cure it. Some of the patients develop complications, but the condition generally does not shorten life. Conventional therapies including anti-histamines for the skin rash, as well as anti-inflammatory drugs, steroids and immunosuppressive drugs for the systemic signs, are usually ineffective. However, the IL-1 receptor antagonist anakinra was found to rapidly control all the symptoms of this syndrome, but signs recur as soon as the treatment is stopped. 

Schnitzler Syndrome Causes

The cause of Schnitzler Syndrome is unknown. Alterations in the cytokine network have been reported. Interleukin-1 alpha binding activity has been described in some patients with Schnitzler Syndrome. Interleukin-1 alpha is a cytokine or inflammatory mediator and could explain some of the inflammatory symptoms of the syndrome. Some of the Schnitzler Syndrome patients have been described with a mutation in the gene NLRP3. Patients with Schnitzler syndrome may present to dermatologists, hematologists, rheumatologists and general physicians because of the variety of possible symptoms. The diagnosis is often delayed because of multiple complications.

Schnitzler Syndrome Symptoms

The signs and symptoms related to the Schnitzler Syndrome may include:

  • Recurrent Hives, mostly on the torso and limbs
  • Typical Onset is at around 55 years old
  • Recurring Fever
  • Joint Pain
  • Bone Pain
  • Muscle Pain
  • Headache
  • Fatigue
  • Weight Loss

Schnitzler Syndrome Clinical Features

Urticaria

Urticaria (hives) describes red raised patches of skin. These are rarely itchy in Schnitzler syndrome, unlike ordinary urticaria. Weals may last longer than ordinary urticaria, often persisting for 12 hours to 3 days. A skin biopsy may or may not show features of urticarial vasculitis, it may show a neutrophil-rich infiltrate. Angioedema (deep swelling) may also occur in some patients. 

Arthritis/ Arthralgia

Painful joints (arthralgia) or swollen joints (arthritis) occur in majority of the Schnitzler Syndrome patients, some patients experience bone pain.

Fever

Intermittent Fever up to 40C affects most of patients with Schnitzler Syndrome, the fever may be accompanied by chills and night sweating.

Organomegaly

Organomegaly refers to enlarged internal organs, i.e., large lymph nodes (lymphadenopathy) in most of  the Schnitzler Syndrome patients, and enlarged liver and spleen (hepato-splenomagaly) in some of the patients.

Hematological Abnormalities

The main hematological (blood) abnormality of Schnitzler Syndrome is monoclonal gammopathy. This usually means a raised level of immunoglobulin M (IgM) but researchers have also reported raised immunoglobulin G (IgG) or combinations of IgM and IgA or IgM and IgG.

Bone marrow tests are normal in majority of the patients at the time of Schnitzler Syndrome diagnosis.

Other Hematological Abnormalities in Schnitzler Syndrome may include:

  • Elevated C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR)
  • Lowered complement levels (C4)
  • Anemia of chronic disease
  • Thrombocytosis (raised platelet count)
  • Neutrophilic Leucocytosis (raised neutrophil white blood cells)

Schnitzler Syndrome Prognosis

Schnitzler syndrome is a chronic condition, and it has not been reported to resolve. Although symptoms can be a nuisance, the condition does not lead to a serious disease in the majority of patients. However some of the patients progress to a lymphoproliferative disorder such as Waldenström macroglobulinemia or B-cell lymphoma. In Schnitzler’s original case the patient died aged 88 years with diffuse “lymphoplasmacytic malignancy” after 20 years of follow up.

Biospecimens

Bay Biosciences is, indeed a global leader in providing researchers with high quality, clinical grade, fully characterized human tissue samples, bio-specimens, and human bio-fluid collections.

Specifically, aamples available include cancer (tumor) tissue, cancer serum, cancer plasma, cancer, peripheral blood mononuclear cells (PBMC) and human tissue samples from most other therapeutic areas and diseases.

Moreover, Bay Biosciences maintains and manages its own biorepository, the human tissue bank (biobank) consisting of thousands of diseased samples (specimens) and from normal healthy donors available in all formats and types.

In fact, our biobank procures and stores fully consented, de-identified and institutional review boards (IRB) approved human tissue samples and matched controls.

Additionally, all our human tissue collections, human specimens and human bio-fluids are provided with detailed, samples associated patient’s clinical data.

In particular, critical patient’s clinical data includes information relating to their past and current disease, treatment history, lifestyle choices, biomarkers, and genetic information.

Moreover, researchers find patient’s data extremely valuable and use it to help identify new effective treatments (drug discovery & development) in oncology, and other therapeutic areas and diseases.

Specifically, Bay Biosciences banks wide variety of human tissue samples and biological samples, including cryogenically preserved at – 80°C.

For example fresh frozen tissue samples, tumor tissue samples, formalin-fixed paraffin-embedded (FFPE), tissue slides, with matching human bio-fluids, whole blood and blood-derived products such as serum, plasma and PBMC.

Furthermore, Bay Biosciences is a global leader in collecting and providing human tissue samples according to the specified requirements and customized, tailor-made collection protocols.

Therefore, please contact us anytime to discuss your special research projects and customized human tissue sample requirements.

Types of Biospecimens

Bay Biosciences provides human tissue samples (human specimens) from diseased and normal healthy donors which includes:

  • Firstly, Peripheral whole-blood
  • Secondly, Amniotic fluid
  • Third, Bronchoalveolar lavage fluid (BAL)
  • Moreover, Sputum
  • Furthermore, Pleural effusion
  • Next, Cerebrospinal fluid (CSF)
  • Also, Serum (sera)
  • Likewise, Plasma
  • In addition, Peripheral blood mononuclear cells (PBMC)
  • For example, Saliva
  • Also, Buffy coat
  • Moreover, Urine
  • Furthermore, stool samples
  • Next, Aqueous humor
  • Likewise, Vitreous humor
  • Lastly, Kidney stones (renal calculi)
  • Finally, Other bodily fluids from most diseases including cancer.

Moreover, we can also procure most human bio-specimens, furthermore; we offer special collections and requests for human samples that are difficult to find. All our human tissue samples are procured through IRB-approved clinical protocols and procedures.

In addition to the standard processing protocols, Bay Biosciences can also provide human plasma, serum, and PBMC bio-fluid samples using custom processing protocols. Additionally you buy donor-specific collections in higher volumes and specified sample aliquots from us.

Furthermore, Bay Biosciences also provides human samples from normal healthy donors; volunteers, for controls and clinical research, contact us Now.

 

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