Adenosine Deaminase Type II Deficiency: Severe Chronic Neutropenia, Lymphoid Infiltration in Bone Marrow, and Inflammatory Features.

Deficiency of adenosine deaminase type 2 (DADA2) is an autoinflammatory disease characterized with immunologic, hematologic, and neurological features. Here, we presented two patients with severe persistent chronic neutropenia, which required differential diagnosis of congenital and autoimmune neutropenia, myelodysplastic syndrome (MDS), and primary immunodeficiency diseases, including autoimmune lymphoproliferative disease. The therapy of the disease except hematopoietic stem cell transplantation is a challenging experience.

Neuroinvasive Listeriosis: Could Petechial Hemorrhages be a Diagnostic Clue?

INTRODUCTION: Listeria monocytogenes-related central nervous system infections may involve the cerebral parenchyma. Meningitis and meningoencephalitis are the most commonly seen forms and mainly affect immunocompromised patients; however, a less frequent form, rhombencephalitis, can occur in otherwise healthy people. Early treatment with appropriate antibiotic therapy is crucial for this otherwise fatal disorder. However, it is not always possible to rapidly establish the diagnosis because of varying presentations and discrepancies in diagnostic tests. CASE REPORT: Herein we report 3 cases of listerial infections involving the central nervous system parenchyma, with versatile diagnostic challenges and related possible solutions and radiologic hints to overcome similar issues in the future. CONCLUSIONS: We point out the importance of nonconventional magnetic resonance imaging techniques in the diagnosis, as we detected petechial hemorrhages in the brain parenchyma in all cases, which can be a diagnostic clue.

Severe measles pneumonia in adults with respiratory failure: role of ribavirin and high-dose vitamin A.

Although measles is usually considered a benign viral disease of childhood, adults may be affected at any age and may experience severe respiratory or neurologic consequences. We present three adult cases (one of whom was pregnant) admitted to our University Hospital who were diagnosed to have measles and who had uncommon clinical features such as hepatitis and hyponatremia. All patients were markedly hypoxic; one required mechanical ventilation. Two patients received therapy with intravenous ribavirin and all patients received high-dose vitamin A for 3 days. Therapy with intravenous ribavirin and vitamin A were well tolerated by our patients except one patient who developed acute renal failure and were associated with reversal of respiratory compromise. Life-threatening measles pneumonitis in adults may be more common than previously appreciated, regardless of the patient's immune status, and ribavirin and high-dose vitamin A might be a treatment option.