WHIM Syndrome – Market Outlook, Epidemiology, Competitive Landscape, and Market Forecast Report – 2023 To 2033


 

WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is an autosomal dominant immune deficiency present from birth, characterized by the abnormal retention of mature neutrophils in the bone marrow (myelokathexis). It can also result in occasional hypogammaglobulinemia, increasing the risk of bacterial infections and susceptibility to human papillomavirus (HPV)-related issues like warts, genital dysplasia, and invasive mucosal carcinoma. The underlying cause of WHIM syndrome is heterozygous gain of function mutations in the CXCR4 gene (2q21), which encodes a chemokine receptor expressed on mature leukocytes. This gene involves several critical processes, including bone marrow cell adhesion, myelopoiesis, and lymphopoiesis. Mutations in CXCR4 lead to prolonged receptor activation, causing the retention of neutrophils and other leukocytes in the bone marrow. WHIM syndrome presents a varied clinical picture, usually in early childhood with recurrent bacterial infections such as pharyngitis, sinusitis, otitis, meningitis, and pneumonia. Vaccination responses are typically poor. More than 80% of patients develop widespread HPV-induced warts, often on the hands and feet, with 25% developing anogenital condylomata acuminata that may progress to intractable dysplastic HPV-induced lesions and invasive genital cancer. Diagnosis relies on clinical signs and specific laboratory findings, including blood cell counts showing neutropenia, lymphopenia, and monocytopenia leading to severe panleukopenia. Serum immunoglobulin G, A, and M levels typically indicate mild hypogammaglobulinemia, and bone marrow aspirates reveal myelokathexis. Genetic analysis of CXCR4 confirms the diagnosis. Current treatment is mainly symptomatic, involving immunoglobulin replacement therapy, prophylactic antibiotics, and granulocyte-colony stimulating factor (GCSF). Standard and more aggressive treatments for HPV-induced lesions often have limited effectiveness. Clinical trials using a CXCR4 antagonist (Plerixafor) are underway, and case reports suggest the HPV vaccine may help reduce HPV infection.

  • WHIM-affected individuals can live into adulthood, but significant risks include intractable dysplastic HPV-induced lesions, invasive genital cancer, and liver failure. By age 40, the cancer risk is approximately 30%.

Thelansis’s “WHIM Syndrome Market Outlook, Epidemiology, Competitive Landscape, and Market Forecast Report – 2023 To 2033" covers disease overview, epidemiology, drug utilization, prescription share analysis, competitive landscape, clinical practice, regulatory landscape, patient share, market uptake, market forecast, and key market insights under the potential WHIM Syndrome treatment modalities options for eight major markets (USA, Germany, France, Italy, Spain, UK, Japan, and China).

KOLs insights of WHIM Syndrome across 8 MM market from the centre of Excellence/ Public/ Private hospitals participated in the study. Insights around current treatment landscape, epidemiology, clinical characteristics, future treatment paradigm, and Unmet needs.

WHIM Syndrome Market Forecast Patient Based Forecast Model (MS. Excel Based Automated Dashboard), which Data Inputs with sourcing, Market Event, and Product Event, Country specific Forecast Model, Market uptake and patient share uptake, Attribute Analysis, Analog Analysis, Disease burden, and pricing scenario, Summary, and Insights.

Thelansis Competitive Intelligence (CI) practice has been established based on a deep understanding of the pharma/biotech business environment to provide an optimized support system to all levels of the decision-making process. It enables business leaders in forward-thinking and proactive decision-making. Thelansis supports scientific and commercial teams in seamless CI support by creating an AI/ ML-based technology-driven platform that manages the data flow from primary and secondary sources.

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