Hematological Findings in Hyper ige Syndrome Patients from Hyderabad, Sindh, Pakistan

Pakistan Journal of Medical & Cardiological Review https://pakjmcr.com/index.php/1/about Online ISSN Print ISSN 3007-2387 3007-2379 Vol. 4 No. 4 (2025) Pakistan Journal of Medical & Cardiological Review Page 394 Hematological Findings in Hyper IgE Syndrome Patients from Hyderabad, Sindh, Pakistan Ali Muhammed Shahani Supervisor PPHI District Laboratory Larkana Naseem Aslam Channa * Professor Institute of Biochemistry University of Sindh Email: [email protected] Dr Sumera Brohi Assistant Professor Obstetrics & Gynecology SMBBMU, CMC Larkana Fawad Ahmed Medical Technologist Liquate Medical and Health sciences Jamshoro Dr Mehak Memon Assistant Prof Department Of Biochemistry, Peoples University of Medical and Health for Women (PUMHSW) Shaheed benazirabad Dr Majida Ali Assistant Professor Obstetrics & Gynecology SMBBMU, CMC Larkana Hyper IgE Syndromes (HIES) are uncommon primary immunological deficits that are typified by high serum IgE levels, eosinophilia, lung infections, recurring eczema, and skin abscesses. Because of their genetic origins, the two types that were first identified as separate illnesses now possess overlapping clinical features but show different symptoms and disease outcomes. Our study involved 100 patients with hyper immunoglobulin E syndrome (HIES) and elevated IgE, randomly selected from hospitals in Hyderabad, alongside 100 matched controls with no Hyper IgE history. A self-structured questionnaire was administered to gather data on sociodemographic characteristics and risk factors. Blood samples were taken for hematological assessment using a Hitachi Cobass 1000 auto-analyzer. Statistical analysis was performed using the student's t-test, ANOVA, and SPSS version 21, with a significance level of p<0.05 at a 95% confidence interval. The study results found that patients with Hyper-IgE Syndrome (HIES) were more likely to be students (37% vs. 19%), predominantly male (57%), and aged 5 to 25 (13%). Symptoms such as shortness of breath, skin rashes, pulmonary infections, and recurring eosinophilia were Abstract Author Details Keywords: Hematological, High IgE, Hyderabad. Received on 15 Sep 2025 Accepted on 05 Oct 2025 Published on 15 Oct 2025 Corresponding E-mail & Author*: PROF. Naseem Aslam Channa * Professor Institute of biochemistry university of Sindh Email: [email protected] Page 395 more prevalent. Chest pain, seasonal allergens, and high fever were associated with HIES, while fast food intake did not affect patient status. Strong correlations were found between bacterial infections and ill health with HIES. Additionally, Complete Blood Count analysis revealed significantly higher levels of neutrophils, lymphocytes, and eosinophils in patients, indicating ongoing inflammation or infection. Study concluded that Patients with Hyper-IgE Syndrome (HIES) are mostly male (57%), aged 5 to 25, and often students (37%). Key symptoms include breathlessness, skin rashes, recurring eosinophilia, fever, seasonal allergies, and chest pain. While fast food does not significantly impact the condition, bacterial infections are linked to poorer health outcomes. Blood tests commonly show elevated neutrophils, lymphocytes, and eosinophils, indicating active infection or inflammation. Introduction The inherited tendency to create IgE antibodies in response to low-dose environmental stimuli, such as dust mites, pollen, and food allergies, is known as atopy. Atopic individuals frequently suffer from food allergies, asthma, atopic dermatitis (AD), and allergic rhinoconjunctivitis. [1, 2] the progression of atopic sickness is shown by allergic march. From birth, atopic infants may respond and become sensitive to allergens. Food allergies are typically detected after AD. Food allergies has a role in the etiology of atopic dermatitis and is present in the majority of its patients. Due to respiratory allergy sensitivity, the table covers bronchial asthma and allergic rhinitis (AR) in later life [3].The most common chronic conditions in children are allergic diseases. According to epidemiological data, allergy-related illnesses are increasing in both industrialized and developing countries as a result of changing environments and lifestyles. [2,4-6]. As a result, further allergy testing is required An early diagnosis aids in the best possible management of allergy disease. Children are first evaluated by primary care physicians. Some children may have signs of conjunctivitis and seasonal AR. Newborns who vomit frequently may have allergies. Choosing who, when, and how to conduct allergy diagnostic testing is part of this process. Hypersensitive reactions to certain allergens that start with immunological processes are known as allergies [7].IgE exacerbates allergic responses and inflammation [8]. Eosinophilia can happen in a number of ways. These consist of helminth infections, atopic diseases, asthma, and drugs. Neoplasms and sensitivities [8]. Eosinophils and IgE have distinct effects on disease. Eosinophilia is a side effect of allergic asthma, which is brought on by IgE. Eosinophil percentage and total immunoglobulin E were assessed in a number of allergy disorders, other hematological disorder may also be related to HIES. Hence present study was aimed to explore the relation of HIES with hematological and to know about the risk factors, we introduce present study pulmonary infection, smoking, Eczematoid, urticarial, breath shortness, severity at night, cough, high fever, chest infection, stress, bacterial infection, poor health, food allergy, meet, fish, egg, dust, and home in polluted area are the risk factors for HIES, whereas, Eosinophilia and Lymphcytopenia are also very common in HIES patients Materials and Methods: Study Design: This cross sectional research ran from January to December of 2024. Five well-known hospitals in Hyderabad and the surrounding areas were selected from their outpatient departments to investigate the origin, 100 (N=100) HIES patients and 100 controls were randomly selected with matched geography and gender after obtaining a signed written informed consent from each participant. A self-structured questionnaire was created Page 396 especially to examine the socio-demographic characteristic and risk factors associated with HIES. Specimen Collection and Questionnaire: In an effort to find patterns linked to HIES, 5ml blood samples taken in an EDTA and yellow top tube. The data was examined for differences in hematological parameters utilizing automated hematology analyzers and immunoassay techniques. A standardized questionnaire was used to collect clinical, demographic, lifestyle, and environmental data from Hyper IgE Syndrome patients in Hyderabad, Sindh, Pakistan, to identify potential risk factors. Ethical Consideration: The University of Sindh, Jamshoro's Ethical Review Committee examined the study protocol, informed consent forms, and questionnaire and approved the study via letter no # IOB/07/2019 dated -03/01/2019 Statistical Analysis The Microsoft Excel 2017 computer program was used to compile and process the data. The risk factors were determined by calculating the odds ratio. The reference was exhibited normal features which indicated OR = 1 (means no association) OR > 1 mean positive association and OR < 1 means negative association with HIES. Results: The comparison of HIES patients and controls in Table 1 revealed that although the age distribution varied slightly, men were more common (57%). 26 to 45 years age group was more prevalent for HIES, students were more in number in HIES to controls. Table 1: Socio-demographic characteristics Characteristics HIES Patients (n=100) Controls (n=100) Gender Male 57 43 Female 43 57 Age Groups 5-25 years 33 28 26-45 years 44 56 46-65 years 18 12 >66 years 5 4 Occupation Housewife 25 21 Student 37 19 Labor 7 7 Child 2 13 Private Employee 10 18 Govt. Employee 20 20 Table 2. Association of some clinical, dietary and environmental factors with HIES are present eosinophilia, pulmonary infections, skin-related symptoms, and shortness of breath among patients and control groups. Patients had a significant association of recurrent eosinophilia, pulmonary infections with HIES, urticarial red skin rashes, and shortness of breath. Furthermore Seasonal allergies, high fever. Bacterial infections and Page 397 poor health were significantly associated with HIES. Whereas, good health was significantly inversely associated with the disease Table 2.0 Association of clinical factors with Hyper Immunoglobulin E Risk factor Patients controls OR(95%CI) P value (<0.05) Recurrent Eosinophilia 60 12 11.00(5.33 -22.689) 0.000 Pulmonary infection 78 21 16.0(8.015-32.092) 0.000 Eczamatoid 12 3 4.4(1.20416.140) 0.029 Urticarial red skin rashes 55 17 5.967 (3.103-11.4790 0.000 Shortness of breath 79 38 6.138(3.27511.503O 0.000 Condition serve at night 87 32 2.719 ( 0,991 -7.456) 0.056 Seasonal allergy, cough 63 16 10.89(5.467 -21.708) 0.000 Condition in Cold 9 9 1.00 (Reference) High fever 70 16 12.25 (6.177-24.293) 0.000 Chest pain 64 15 51.3(18.864-139.892) 0.000 Stress 55 20 4.88 (2.60 – 9.16 0.537 Fast food 34 46 0.605(0.342-1.070) 0.112 Bacterial infection 13 5 4.481(1.503-13.357) 0.009 Poor health 48 3 14.08(4.096 -48.401) 0.00 Good health 2 47 0.037(0.009-0.163) 0.000 A substantial association Patients with elevated IgE levels and the disease, possibly contributing to its pathophysiology, is suggested by the data presented in Figure 1.which shows a significant difference in IgE levels between the case and control groups, with the case group having significantly higher levels at 1069 IU/ML were included in present study Figure 1.0 shows the IgE level 1069 70 0 200 400 600 800 1000 1200 Case Control Con: (IU/ML) Case and Control Page 398 In table 3.0, we analyzed the hematology of the patients and controls and noted variations. In patients the red blood cell count was somewhat greater, and the hematocrit and hemoglobin levels were slightly higher. Hemoglobin concentration and mean corpuscular volume did not differ significantly. The case group showed slightly higher WBC counts than controls, but no significant difference. All the other Platelet counts were similar, but differential counts showed significant immune cell profile deviations. Neutrophils, lymphocytes, and eosinophils were significantly elevated in cases compared to controls, indicating an ongoing inflammatory or infectious process. These findings suggest immune system activation or response, possibly due to allergies or eosinophilic components. The data suggests that neutrophil, lymphocyte, and eosinophil levels may serve as key hematologic indicators in the patient group, despite general blood parameters remained stable. Table 3.0 Comparison of Complete Blood Count (CBC) Parameters between Cases and Controls Discussion: As indicated in table -1 male patients are prominently higher, Another study found a male predominance among Hyper-IgE Syndrome (HIES) patients (57%), indicating a higher as incidence of primary immune deficiencies in males due to X-linked genetic patterns [9]. Our study also found a significant presentation 26-45 years age group in the patient group (44%) compared to controls (56%), which is in contrast with Pakistan's general pediatric admission trends. This could be due to delayed diagnosis or lack of awareness [10]. Our study found a high proportion of students among HIES patients (37%), possibly due to adolescent symptoms or access to educational environments have worsen their condition (table-1) due to allergy. Other Pakistan study shows the informal sector workers, laborers, and agricultural workers are more affected by health disparities due to poor working conditions and limited care access, which is not comparable with present study [11]. The study reveals that patients with Hyper-IgE Syndrome HIES in Pakistan have a higher prevalence of recurrent eosinophilia, pulmonary infections, eczematoid conditions, urticarial red skin rashes, and shortness of breath compared to controls (table -2) Symptoms are more pronounced at night, and seasonal allergies and high fever are strongly associated with the condition [12]. Global studies on HIES consistently show elevated eosinophil counts, increased pulmonary infection susceptibility, skin conditions like eczema and urticaria, nocturnal CBC parameter Case (n=100) Mean ± SD Controls (n= 100) Mean ± SD p-Value <0.05 RBCs 4.85±5.36 4.62±0.64 0.007 WBCs 10.32±9.77 9.58±4.33 0.49 Platelets 286.16±99.66 284.75±135.31 0.93 Haemoglobin 12.72±1.87 12.43±2.07 0.29 Heamotocrit 39.72±4.80 38.45±5.36 0.08 MCV 82.50±8.39 83.37±8.89 0.47 MCH 26.49±3.28 27.02±3.11 0.29 MCHC 35.08±31.32 32.33±2.11 0.38 Neutrophils 59.78±13.95 54.36±13.17 0.005 Lymphocytes 34.93±10.80 27.69±12.70 0.007 Monocytes 7.40±2.31 7.89±3.81 0.30 Eosinophls 4.40±4.21 2.82±2.69 0.001 Basophils 0.47±0.27 0.40±0.25 0.07 Page 399 exacerbation, seasonal allergies, and high fever in HIES patients [13]. Our study on primary immune deficiencies in Pakistan aligns with previous research, but the higher prevalence of certain symptoms may indicate regional variations in healthcare access and diagnostic capabilities [14]. The study's findings suggest that genetic and immunological factors are more significant in the pathogenesis of HIES, despite the lack of significant association with stress and dietary factors. Our study analyzes hematological parameters in patients with Hyper-IgE Syndrome, revealing elevated WBC counts, suggesting an active immune response, potentially indicating inflammation or allergic reactions.( Taable-3) In comparison with other study Batool et al.'s 2022 study found leukocytosis in 46.2% of pulmonary tuberculosis patients, with elevated WBC counts, but further research is needed to understand differential leukocyte counts in HIES patients [15]. Another study Mazhar et al.'s 2021 study found elevated WBC counts in healthy adults across Pakistan, suggesting they may serve as potential biomarkers for immune system activation in HIES patients [16]. Akhtar et al.'s 2022 study found thrombocytopenia in 34.1% of aplastic anemia patients, while our study found stable platelet counts in HIES patients, contrasting their hematological profiles [17]. CONCLUSION: This study concludes that many immunological deficits have a hereditary foundation due to the frequency of Hyper-IgE Syndrome (HIES) in Pakistan, which is shown to be male (57%). The underrepresentation of youngsters (2%) is alarming because it could be the result of ignorance or a delayed diagnosis. A higher likelihood of symptom onset during adolescence or simpler access to healthcare may be indicated by the high percentage of students (37%) among HIES patients. Shortness of breath, skin disorders, lung infections, and recurring eosinophilia are some of the main characteristics of HIES. Stable platelet levels contrast with thrombocytopenia in situations of aplastic anemia, but elevated WBC numbers indicate continued immunological activation. The study emphasizes the need for better pediatric screening, more diagnostic awareness, and a better comprehension of the immunological underpinnings of the illness. References: Borish L. Allergic rhinitis: systemic inflammation and implications for management. J Allergy Clin Immunol. 2003 Dec;112(6):1021-31. Thomsen SF. Epidemiology and natural history of atopic diseases. Eur Clin Respir J. 2015 Mar 24;2. Spergel JM. From atopic dermatitis to asthma: the atopic march. Ann Allergy Asthma Immunol. 2010 Aug;105(2):99-106; quiz 107-9, 117. Eigenmann, P. A., Atanaskovic‐Markovic, M., O'B Hourihane, J., Lack, G., Lau, S., Matricardi, P. M., ... & Høst, A. (2013). Testing children for allergies: why, how, who and when: an updated statement of the European Academy of Allergy and Clinical Immunology (EAACI) Section on Pediatrics and the EAACI‐Clemens von Pirquet Foundation. Pediatric allergy and immunology, 24(2), 195-209. Holgate ST, Lack G. Improving the management of atopic disease. Arch Dis Child. 2005 Aug;90(8):826-31. Al-Mughales JA. Diagnostic Utility of Total IgE in Foods, Inhalant, and Multiple Allergies in Saudi Arabia. J Immunol Res. 2016;2016:1058632. Arshad SH, Tariq SM, Matthews S, Hakim E. Sensitization to common allergens and its association with allergic disorders at age 4 years: a whole population birth cohort study. Pediatrics. 2001 Aug;108(2):E33. Froidure A, Mouthuy J, Durham SR, Chanez P, Sibille Y, Pilette C. Asthma phenotypes and IgE responses. Eur Respir J. 2016 Jan;47(1):304-19. Page 400 Chen, J. Y., Cortes, C., & Ferreira, V. P. (2018). Properdin: A multifaceted molecule involved in inflammation and diseases. Molecular immunology, 102, 58-72. Wallace, M. R., Hale, B. R., Utz, G. C., Olson, P. E., Earhart, K. C., Thornton, S. A., & Hyams, K. C. (2002). Endemic infectious diseases of Afghanistan. Clinical Infectious Diseases, 34(Supplement_5), S171-S207. Saheed, R., Ahmad, N., & Rahim, M. T. (2021). Income Inequalities among Earners Engaged in Selected Occupations and Professions in Pakistan. Global Economics Review, VI, 103-120. Qureshi, S., Mir, F., Junejo, S., Saleem, K., Zaidi, S., Naveed, A. B., ... & Qamar, F. N. (2020). The spectrum of primary immunodeficiencies at a tertiary care hospital in Pakistan. World Allergy Organization Journal, 13(7), 100133 Minegishi, Y. (2021). Hyper-IgE syndrome, 2021 update. Allergology International, 70(4), 407-414. Saikia, B., Rawat, A., Minz, R. W., Suri, D., Pandiarajan, V., Jindal, A., ... & Singh, S. (2021). Clinical profile of hyper-IgE syndrome in India. Frontiers in Immunology, 12, 626593. Batool, Y., Pervaiz, G., Arooj, A., & Fatima, S. (2022). Hematological manifestations in patients newly diagnosed with pulmonary tuberculosis. Pakistan Journal of Medical Sciences, 38(7), 1968. Mugheri, I. A., Ansari, I. T., Qadir, A., Shaikh, R., Saber, M., & Mugheri, A. J. (2025) Assessment of platelet count and its association with stroke severity in patients with acute ischemic stroke from larkana. Review Journal of Neurological & Medical Sciences Review, 3(4), 709-714. Akhtar, A., Ahmad, R., Chaudhary, S. S., Kaleemi, S., Saeed, N., & Danish, M. (2022) Severity and clinico-haematological features of aplastic anemia in adults presenting to public sector tertiary care hospital of lahore: a descriptive crosssectional study. JAIMC, 20(3), 156-160.