Patients will receive a pre-transplant conditioning regimen of Busulfan Fludarabine and Alemtuzumab. For patients with pre-transplant hepatic dysfunction, Melphalan will be substituted for the Busulfan. For patients receiving a second transplant or a "boost", pre-transplant conditioning based on the clinical condition of the patient will be determined by the Principal Investigator and the patient's bone marrow transplantation (BMT) physician. The donor peripheral blood stem cells will undergo CD34+ selection (Biological/Vaccine: CD34 Stem Cell Selection Therapy). The CliniMACS (PLUS) Reagent System will be used to remove T-cells from the peripheral blood stem cell transplant in order to decrease the risk of acute and chronic graft versus host disease (GVHD).

Bone Marrow Failure Syndrome

Severe Aplastic Anemia

Severe Congenital Neutropenia

Amegakaryocytic Thrombocytopenia

Diamond-Blackfan Anemia

Schwachman Diamond Syndrome

Primary Immunodeficiency Syndromes

Acquired Immunodeficiency Syndromes

Histiocytic Syndrome

Familial Hemophagocytic Lymphocytosis

Lymphohistiocytosis

Macrophage Activation Syndrome

Langerhans Cell Histiocytosis (LCH)

Hemoglobinopathies

Sickle Cell Disease

Sickle Cell-beta-thalassemia

The CliniMACS (PLUS) Reagent System (Miltenyi CliniMACS CD34+ Cell Selection Device) will be used to remove T-cells from the peripheral blood stem cell transplant in order to decrease the risk of acute and chronic graft versus host disease (GVHD).

CD34 Stem Cell Selection Therapy

Morgan Stanley Children's Hospital, New York-Presbyterian, Columbia University

CD34+ (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation

CD34+ Stem Cell Selection for Patients Receiving a Matched or Partially Matched Family or Unrelated Adult Donor Allogeneic Stem Cell Transplantation for Non-Malignant Disease

Determine the incidence and severity of acute GVHD.

Quantify the incidence of primary and secondary graft failure.

To assess event free survival and overall survival

To determine the time to neutrophil and platelet engraftment

To determine the time to immune reconstitution (including normalization of T, B and NK cell repertoire and Immunoglobulin G production)

To establish the incidence of infectious complications including bacterial, viral, fungal and atypical mycobacterial and other infections

hemoglobinopathies/diarreia

CD34+ (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation

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