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Whipple Disease

Updated : September 17, 2022





Background

Tropheryma whippelii is a gram-positive bacterium that causes Whipple illness. This is really a systemic condition that affects the cardiovascular, CNS, vascular systems, and joints in addition to the gastrointestinal tract’s malabsorption. This syndrome was first explained by Dr. George Hoyt Whipple in 1907.

He received the Physiology Nobel Prize as the first American. Dr. Whipple discussed the example of a thirty-six-year-old man who had skin pigmentation, arthralgias, and mesenteric lymphadenopathy in addition to malabsorption.

His report was posted in the Johns Hopkins Hospital Bulletin, and he gave the condition the term “Intestinal Lipodystrophy.” Although he postulated infectious organisms as the cause, the bacteria weren’t properly discovered until 1992. Whipple disease is uncommon, and the literature solely contains case accounts.

Epidemiology

Whipple syndrome is a rare ailment in the world, with the majority of reports coming from Europe and North America. The HLA B27 haplotype is connected to the illness. Around one to three persons out of every million are affected by Whipple illness globally.

The average age at which symptoms first appear is 55. With such a ratio of 4:1, men experience it significantly more frequently than women do. The increasing occurrence in farmers is likely due to the organism’s apparent soil habitation.

Anatomy

Pathophysiology

Although the precise pathophysiology is yet unknown, there is enough data to suggest that host immunity plays a significant role. The majority of people who get T. whippelii are asymptomatic carriers or only have a mild illness, which is followed by the emergence of cellular and humoral defenses.

The immune response to the organism is less in sick people. It mostly comprises type 1 T-cell reaction that is compromised as well as impaired macrophage activation and activity. Shigella flexneri and Streptococcal groups B and G are antigenically similar to Whipple bacillus.

The 2- to 3-fold rise in HLA-B27 antigen frequency across individuals affected suggests that host factors play a significant role in the pathogenesis. The macrophages that can be seen in periodic acid-Schiff consume the creature. Sadly, Whipple disease cannot be diagnosed by looking for periodic acid-Schiff-stained macrophages.

Coccobacillus particles that refract the species may be seen under an electron microscope. The natural villus activity has been disturbed, which results in the observed malabsorption. The microorganism can be discovered in numerous tissues whenever systemic illness develops.

Etiology

1992 saw the description of Tropheryma whipplei. It was given the name Tropheryma whippelii up till 2001 when its name was changed to reflect Dr. George Hoyt Whipple’s correct spelling. It is an acid-fast negative, periodic acid-Schiff positive (PAS), and gram-positive bacillus.

The bacillus core is encased in a plasma membrane, which is encircled by a three-layered cell wall, according to electron microscopy findings. Polysaccharides that PAS positively stain are present inside the inner lining.

The host’s inability to produce a humoral antibody production towards the infection may be explained by the outermost layer, which mimics a plasma membrane and could be of host source.

Genetics

Prognostic Factors

With quick identification and management of Whipple’s illness, the prognosis is typically favorable. Between 2 to 3 weeks of starting the treatment, the patients report feeling significantly better. Patients who are not treated have a dismal prognosis.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK441937/

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Whipple Disease

Updated : September 17, 2022




Tropheryma whippelii is a gram-positive bacterium that causes Whipple illness. This is really a systemic condition that affects the cardiovascular, CNS, vascular systems, and joints in addition to the gastrointestinal tract’s malabsorption. This syndrome was first explained by Dr. George Hoyt Whipple in 1907.

He received the Physiology Nobel Prize as the first American. Dr. Whipple discussed the example of a thirty-six-year-old man who had skin pigmentation, arthralgias, and mesenteric lymphadenopathy in addition to malabsorption.

His report was posted in the Johns Hopkins Hospital Bulletin, and he gave the condition the term “Intestinal Lipodystrophy.” Although he postulated infectious organisms as the cause, the bacteria weren’t properly discovered until 1992. Whipple disease is uncommon, and the literature solely contains case accounts.

Whipple syndrome is a rare ailment in the world, with the majority of reports coming from Europe and North America. The HLA B27 haplotype is connected to the illness. Around one to three persons out of every million are affected by Whipple illness globally.

The average age at which symptoms first appear is 55. With such a ratio of 4:1, men experience it significantly more frequently than women do. The increasing occurrence in farmers is likely due to the organism’s apparent soil habitation.

Although the precise pathophysiology is yet unknown, there is enough data to suggest that host immunity plays a significant role. The majority of people who get T. whippelii are asymptomatic carriers or only have a mild illness, which is followed by the emergence of cellular and humoral defenses.

The immune response to the organism is less in sick people. It mostly comprises type 1 T-cell reaction that is compromised as well as impaired macrophage activation and activity. Shigella flexneri and Streptococcal groups B and G are antigenically similar to Whipple bacillus.

The 2- to 3-fold rise in HLA-B27 antigen frequency across individuals affected suggests that host factors play a significant role in the pathogenesis. The macrophages that can be seen in periodic acid-Schiff consume the creature. Sadly, Whipple disease cannot be diagnosed by looking for periodic acid-Schiff-stained macrophages.

Coccobacillus particles that refract the species may be seen under an electron microscope. The natural villus activity has been disturbed, which results in the observed malabsorption. The microorganism can be discovered in numerous tissues whenever systemic illness develops.

1992 saw the description of Tropheryma whipplei. It was given the name Tropheryma whippelii up till 2001 when its name was changed to reflect Dr. George Hoyt Whipple’s correct spelling. It is an acid-fast negative, periodic acid-Schiff positive (PAS), and gram-positive bacillus.

The bacillus core is encased in a plasma membrane, which is encircled by a three-layered cell wall, according to electron microscopy findings. Polysaccharides that PAS positively stain are present inside the inner lining.

The host’s inability to produce a humoral antibody production towards the infection may be explained by the outermost layer, which mimics a plasma membrane and could be of host source.

With quick identification and management of Whipple’s illness, the prognosis is typically favorable. Between 2 to 3 weeks of starting the treatment, the patients report feeling significantly better. Patients who are not treated have a dismal prognosis.

https://www.ncbi.nlm.nih.gov/books/NBK441937/

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