CPHON Bone Marrow Transplant and Stem Cell Therapy 1

Question 1

A pediatric patient undergoing allogeneic hematopoietic stem cell transplant (HSCT) develops a skin rash, diarrhea, and elevated bilirubin 3 weeks post-transplant. The nurse recognizes this as which complication?

Accepted Answer

Acute graft-versus-host disease (aGVHD)

Answer

Acute GVHD classically presents within 100 days of allogeneic HSCT with involvement of skin (maculopapular rash), GI tract (diarrhea, cramping, nausea), and liver (elevated bilirubin, alkaline phosphatase).

Question 2

Which conditioning regimen used prior to HSCT is associated with the highest risk of veno-occlusive disease (VOD)/sinusoidal obstruction syndrome?

Accepted Answer

Myeloablative conditioning with busulfan and cyclophosphamide

Answer

High-dose busulfan combined with cyclophosphamide in myeloablative conditioning regimens causes the most significant hepatic sinusoidal endothelial cell injury, leading to VOD/SOS.

Question 3

What is the primary purpose of the preparative (conditioning) regimen given before HSCT?

Accepted Answer

To eradicate residual malignancy and create marrow space for donor cells

Answer

The conditioning regimen serves the dual purpose of destroying residual malignant cells (eradication) and creating immunosuppression and physical space in the bone marrow to allow engraftment of donor hematopoietic stem cells.

Question 4

A child 6 months post-allogeneic HSCT develops dry eyes, oral mucosal changes, joint contractures, and skin tightening. This clinical picture is most consistent with:

Accepted Answer

Chronic graft-versus-host disease (cGVHD)

Answer

Chronic GVHD is a multi-system inflammatory and fibrotic disorder occurring after 100 days post-transplant, resembling autoimmune connective tissue diseases with features of scleroderma, Sjögren's, and lichen planus.

Question 5

What is engraftment, and what laboratory finding confirms that it has occurred following HSCT?

Accepted Answer

Recovery of donor-derived hematopoiesis, confirmed by absolute neutrophil count (ANC) ≥500/µL for 3 consecutive days

Answer

Engraftment is defined as the successful establishment of donor hematopoietic stem cells in the recipient's bone marrow, with the primary indicator being an ANC of ≥500/µL (some use ≥500 for 3 days or ≥1000 for 1 day).

CPHON Exam Practice Test

CPHON Exam Practice Test

CPHON Bone Marrow Transplant and Stem Cell Therapy 1