**7. Prognosis**

*Congenital Anomalies in Newborn Infants - Clinical and Etiopathological Perspectives*

Gradually, the cellular elements in the thymus and lymphatic organs begin to disappear [27]. Depletion of small lymphocytes from T cell areas, reticular cell stroma protrusions, and the presence of abnormal plasma cells, often associated with extracellular plasmacytosis and hematopoiesis, are consistently seen in the lymph nodes and spleen of patients with WAS [28]. In a study of spleens obtained from WAS patients undergoing splenectomy, gradual depletion of the marginal zone involving B cells was also observed [29]. It may be possible to justify the disruption of the antibody response to selected polysaccharides and protein antigens

WAS is an X-linked disease presented in males, with a lack of clinical symptoms in female carriers. Also, a deleterious mutation of the paternally derived X chromosome and inactivation of the maternally derived X chromosome lead to WAS in females, which is rare [30]. The diagnosis of WAS should be conducted in any male appearing with thrombocytopenia, eczema, recurrent respiratory infections, autoimmunity, etc (**Table 2**). Clinical findings may or may not be present during the course of the disease. Therefore, Because of evolution in clinical, physical, and

Bleeding Mucosal bleeding (easy bruising, epistaxis, hematochezia, hematuria) or intracranial hemorrhage Infections Recurrent or severe sinopulmonary infections, viral infections, fungal infections, or opportunistic infections

Autoimmunity Cytopenias, vasculitis, inflammatory bowel disease, arthritis, renal

Complete blood cell count Anemia, microcytosis, thrombocytopenia, low mean platelet volume

protein, polysaccharide, and conjugate vaccines

Abnormal isohemagglutinin titers and diminished vaccine responses to

T-cell lymphopenia and abnormal proliferative responses to mitogens

X-linked disorder Every generation affected; predominant male susceptibility

disease

laboratory findings, there is a dire need for Reassessment over time [5].

**5. Histopathology**

**6. Diagnosis**

**Physical exams**

Past medical history

Family history

Laboratory exam

titers

Isohemagglutinin and vaccine

Lymphocyte subsets and mitogen responses

*Abbreviation: Ig, immunoglobulin.*

Rash Eczema

Rash Eczema

Malignancy Lymphoma

Peripheral blood smear Microthrombocytes Serum IgG, IgA, IgM, and IgE Low lgG, lgA, lgM; high lgE

*Clues to diagnosis of Wiskott–Aldrich syndrome [acc.6].*

Bleeding Petechiae, ecchymoses

by examining these histological abnormalities.

**186**

**Table 2.**

The prognosis of X-linked thrombocytopenia has got a favorable position with a life expectancy close to the normal population [25]. Whereas, Classic Wiskott Aldrich syndrome has a poor prognosis with decreased life expectancy, which can be attributed to recurrent infections, autoimmune disease, and malignancy. Hemorrhage is the main cause of death in these patients [5].

As soon as a diagnosis is confirmed, the patient should be monitored and evaluated in a center with expertise in the management of WAS. It is clear that Without appropriate care and intervention, morbidity and mortality will not be unexpected. In a retrospective research, it has been demonstrated 36% of patients with WAS experienced non-HSCT-associated deaths at a mean age of 8 years. Mainly, these deaths are caused by infection (44%), bleeding (23%), and malignancy (26%) [5].

Many centers that provide look after WAS patients have multifaceted approaches to the care of patients and affected members of family, including genetic counseling, psychosocial support services, and subspecialist support, like transplant immunology, hematology/oncology, communicable disease, and important care. Of note, with appropriate care and timely intervention, WAS patients have an interesting prognosis. As an example, long-term survival following the utilization of allogeneic HSCT is >80% [31].
