Publications by authors named "Sujoy Khan"

A case of a 28-year-old woman with known C1-inhibitor deficiency (functional, Type II) with persistent bilateral non-pruritic, mildly photosensitive facial rash for 10 months following delivery of her second child is presented. Histology of the skin was suggestive of tumid lupus erythematosus (LE), but no other features of systemic LE (ANA, dsDNA negative) were evident. She had stopped danazol which was controlling the underlying disease, and once this was restarted and treatment for tumid lupus was started, she improved.

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Article Synopsis
  • Hereditary angioedema (HAE) is a rare genetic disorder leading to recurrent swelling, which can be life-threatening, and its treatment includes both on-demand and preventive options.
  • The last UK consensus on HAE was in 2014, but new medications have emerged, and there's a call for long-term prophylaxis (LTP) to effectively manage the condition and enhance patient quality of life.
  • A recent Delphi process revealed that current UK access criteria for LTP based solely on attack frequency are too simplistic and may overlook patients who could benefit, emphasizing the need for expert care and improved monitoring practices.
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Chronic lymphocytic leukaemia (CLL) is a malignancy of clonally expanded antigen-switched, neoplastic, mature B cells. CLL is characterised by a variable degree of immunosuppression and secondary hypogammaglobulinemia. B-cell depleting therapies have historically been deployed with a proportion of patients becoming resistant to multiple lines of treatment with an associated worsening of immunosuppression and heightened infection risk.

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Background: Chronic spontaneous urticaria (CSU) can be extremely debilitating to the patient and challenging for the treating clinician. The National Institute of Health and Clinical Excellence (NICE) in the United Kingdom (UK) recommendation of omalizumab for patients who fail to respond to high-dose anti-histamines has improved treatment options and quality of life. However, there is still lack of clear guidelines for treatment of patients resistant to standard and anti-IgE therapies.

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Background: Individuals with primary and secondary immunodeficiency (PID/SID) were shown to be at risk of poor outcomes during the early stages of the SARS-CoV-2 pandemic. SARS-CoV-2 vaccines demonstrate reduced immunogenicity in these patients.

Objectives: To understand whether the risk of severe COVID-19 in individuals with PID or SID has changed following the deployment of vaccination and therapeutics in the context of the emergence of novel viral variants of concern.

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Article Synopsis
  • In March 2020, the UK Primary Immunodeficiency Network created a registry to track outcomes of individuals with Primary Immunodeficiency Diseases (PID) and Secondary Immunodeficiency Diseases (SID) after infection with SARS-CoV-2, reporting 310 cases.
  • The overall mortality rate was 17.7%, with certain groups experiencing higher rates; for example, Common Variable Immunodeficiency Disease (CVID) had an infection fatality rate (IFR) of 18.3%.
  • Individuals with PID and SID faced greater risks in terms of inpatient mortality and generally died at younger ages than the broader population, with risk factors including older age, low lymphocyte count before infection, and existing co-morbidities
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Bacterial infections are a major cause of morbidity and mortality in chronic lymphocytic leukemia (CLL), and infection risk increases in patients treated with the Bruton's tyrosine kinase (Btk) inhibitor, ibrutinib. Btk and related kinases (like Tec) are expressed in non-leukemic hematopoietic cells and can be targeted by ibrutinib. In platelets, ibrutinib therapy is associated with bleeding complications mostly due to off-target effects.

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Background: Patients with some types of immunodeficiency can experience chronic or relapsing infection with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). This leads to morbidity and mortality, infection control challenges, and the risk of evolution of novel viral variants. The optimal treatment for chronic coronavirus disease 2019 (COVID-19) is unknown.

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