Primary immunodeficiency diseases (PIDs) are a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins, natural killer cells, and T and B lymphocytes. Congenital defect in phagocyte number and functions are about 20% of all primary immunodeficiency. Some of these illness present as neutropenic patient.In severe congenital NEUTROPENIA myeloid differentiation is failed and there are 3 types gene defect in ELA2 (mistrafficking of elastase. repression of elastase. G-CSFR) all types in herited as autosomal dominant.Kostmann disease has NEUTROPENIA with defect in HAX1 gene that control apoptosis of neutrphil and inherited as autosomal recessive. In cyclic NEUTROPENIA due to mistrafficking of elastase (ELASTASE 2) there is oscillation in neutrophils. P 14 deficiency is autosomal recessive disorder with hypogammaglubolinemia and NEUTROPENIA. Shwachman-Diamond Syndrome presented as pancreatic exocrine deficincy with NEUTROPENIA.There are some primary immunodeficincy who NEUTROPENIA is a part of paraclinical presentation such as some primary humoral immunodeficincy. There is common clinical presentation in neutropenic patients, skin infections, mucosal involement in infectious diseases, lung infections with some common micro organism such as staph aureus, gram negative organism and fungi are some of these similarity.

Purpose: To present a 1-year-old boy with cyclic NEUTROPENIA who presented with multiple episodes of periorbital cellulitis (POC). Methods: The child presented with three episodes of POC. In the second episode, the cellulitis was associated with nasolacrimal duct obstruction, and in the third episode, a pansinusitis was noted. He underwent a thorough systemic evaluation. Results: Patient’ s evaluation revealed the diagnosis of cyclic NEUTROPENIA. Conclusion: This report emphasizes the possibility of an underlying immunodeficiency with recurrent POC, even in an apparently healthy child.

NEUTROPENIA is characterized by decrease in the absolute number of circulating neutrophils and an increase susceptibility to infections. The current study was performed in order to explain the clinical and laboratory findings of patients with antibody deficiency disorders associated NEUTROPENIA. The patients records of 19 neutropenic cases out of 207 patients with antibody deficiencies, who had been referred to Childrens Medical Center and enrolled in Iranian primary immunodeficiency registry, were reviewed. Nineteen cases (14 male and 5 female), with a mean age of 10.7±5.7 years, were associated with NEUTROPENIA (9.2%). The disorders with associated NEUTROPENIA were Hyper IgM syndromes (3 of 8), Common variable immunodeficiency (13 of 109), and X-linked agammaglobulinemia (3 of 45). The median age for the onset of disease and diagnosis age were 15 months (1-134) and 3.8 years (6 months-13 years), respectively. The most common infections during the course of illness were pneumonia (13 cases), diarrhea (12 cases), oral candidiasis (9 cases), otitis media (6 cases), sinusitis (6 cases), cutaneous infections (5 cases), and abscess (5 cases). Other less frequent infections were: conjunctivitis, oral ulcers, meningitis, and osteomyelitis. Three neutropenic patients died because of recurrent infections. NEUTROPENIA may occur in any of the primary immunodeficiency disorders. Persistent or severe infections always pose a supposition, which deserves further evaluation for detecting an underlying immune deficiency syndrome and NEUTROPENIA, since a delay in diagnosis may result in a serious organ damage or even death of the patient.

Clozapine is known as one of the atypical antipsychotics which is placed in the second line of medical treatment for schizophrenia due to its hematologic complications. It is used in cases of resistance to treatment. Some side effects of clozapine include leukopenia, granulocytopenia, fever, hepatotoxicity, sedation, dizziness, hypotension, weight gain, constipation, and seizure. NEUTROPENIA and hepatotoxicity have been separately reported after taking atypical antipsychotics, including clozapine. However, simultaneous occurrence of these two complications is rare and has not been reported with clozapine use. This study reports a case of concurrent hepatotoxicity and NEUTROPENIA induced by clozapine. The patient was a 58-year-old man who started taking clozapine for the first time in March 2017, about seven weeks before his recent admission, because of a history of treatment-resistant schizophrenia. He had been referred to the emergency department of a general hospital with symptoms of weakness, lethargy, fever, and chills. The laboratory results showed NEUTROPENIA with a frequency of 352 × 103 (17. 5%) and hepatotoxicity with alanine transferase (ALT) = 139 u/L, aspartate transferase (AST) = 214 u/L, total bilirubin = 11. 5 mg/dL, and direct bilirubin = 9. 3 mg/Dl, caused by taking clozapine. The symptoms were attenuated within eight days after discontinuation of clozapine. Moreover, the patient’ s para-clinical complications including NEUTROPENIA, and raised transaminases and bilirubin returned to normal. It was concluded that clozapine can simultaneously cause NEUTROPENIA and hepatotoxicity; physicians are recommended to be aware of this issue to prevent mortality through appropriate and timely diagnosis.