A 22-year-old woman is evaluated for fever, diarrhea, and skin rash. The patient was admitted 10 days ago after a motor vehicle collision resulted in a splenic laceration. She underwent an emergency splenectomy and received multiple units of emergency-release type O-negative blood perioperatively. Postoperatively, she received the pneumococcal vaccine but not the meningococcal vaccine as it had been given 2 years ago. The patient had been recovering well with no issues until 2 days ago when she developed a nonpruritic skin rash on her trunk that progressed to involve the face and extremities. She has also had fevers as high as 38.8 C (102 F) and profuse, watery diarrhea. The patient has a history of Hodgkin disease and received her most recent dose of chemotherapy 3 weeks ago.
New findings on the physical examination compared to 2 days ago include a diffuse maculopapular skin rash sparing the palms and soles and mild, nontender hepatomegaly.
Laboratory test results are as follows:
Stool Clostridium difficile testing is negative.
Which of the following would have been most effective in preventing this patient's current condition?

Question

A 22-year-old woman is evaluated for fever, diarrhea, and skin rash.  The patient was admitted 10 days ago after a motor vehicle collision resulted in a splenic laceration.  She underwent an emergency splenectomy and received multiple units of emergency-release type O-negative blood perioperatively.  Postoperatively, she received the pneumococcal vaccine but not the meningococcal vaccine as it had been given 2 years ago.  The patient had been recovering well with no issues until 2 days ago when she developed a nonpruritic skin rash on her trunk that progressed to involve the face and extremities.  She has also had fevers as high as 38.8 C (102 F) and profuse, watery diarrhea.  The patient has a history of Hodgkin disease and received her most recent dose of chemotherapy 3 weeks ago.
New findings on the physical examination compared to 2 days ago include a diffuse maculopapular skin rash sparing the palms and soles and mild, nontender hepatomegaly.
Laboratory test results are as follows:
 Stool Clostridium difficile testing is negative.
Which of the following would have been most effective in preventing this patient's current condition?

Quizplus · Accepted Answer

This patient with Hodgkin disease underwent emergency blood transfusion after a motor vehicle collision and then, several days later, developed fever, rash, profuse diarrhea, and lymphopenia. This chronology raises strong suspicion for transfusion-associated graft-versus-host disease (ta-GVHD), a rare condition that occurs when donor lymphocytes from transfused blood attack host tissue. Nearly all cases arise in patients who are immunocompromised (eg, Hodgkin lymphoma with recent chemotherapy) or those who are a partial HLA match to the donor (eg, family member). Manifestations typically occur 4-30 days after transfusion and include fever and an erythematous/maculopapular rash that often becomes generalized; additional features include anorexia, (right upper-quadrant) abdominal pain, hepatomegaly, liver function abnormalities, and profuse water diarrhea (>7-8 L/day). Unlike GVHD associated with hematopoietic stem cell transplantation, ta-GVHD causes bone marrow destruction leading to progressive pancytopenia, which is the major underlying cause of morbidity and death. Treatment is typically ineffective, and most cases (80%-90%) are fatal. Therefore, prevention is paramount. Those at high risk for the condition (eg, immunocompromised state) should receive irradiated blood products to inactivate donor lymphocytes prior to transfusion. Patients with HIV do not appear to be at risk for this condition and can receive nonirradiated blood (possibly because HIV alters donor lymphocyte survival). (Choice A) Cytomegalovirus (CMV) can be transmitted via transfused blood and typically causes a mononucleosis-like syndrome, which can include fever, maculopapular rash, liver function abnormalities, and systemic symptoms. However, most patients also have absolute lymphocytosis and atypical lymphocyte cells on peripheral smear. CMV blood screening is typically required prior to transfusion for CMV-seronegative patients who have HIV, organ transplantation, or hematopoietic cell transplantation (alternately, they can receive leuko-reduced blood). (Choice B) Patients with asplenia are at elevated risk for meningococcal disease, which can cause high fever, nonspecific symptoms, and diffuse petechial or hemorrhagic rash. However, most patients develop headache, stiff neck, and sepsis within 12-15 hours of symptom onset. The booster vaccine for meningococcus is usually not required until the 5th year after initial vaccination (in those with asplenia). (Choice C) Transfusion of ABO-incompatible erythrocytes typically leads to a severe, life-threatening reaction (eg, fevers, chills, hypotension, acute hemolytic anemia, renal insufficiency) within minutes of transfusion initiation. This patient received O-negative blood and had delayed manifestations, making ABO incompatibility unlikely. (Choice D) Washing red blood cells removes the small amount of residual plasma from the preparation and is used for patients with severe/recurrent allergic transfusion reactions (eg, hives) or those with IgA deficiency. Educational objective: Patients who are immunodeficient or those receiving blood products from a family donor or HLA-matched platelets should receive irradiated blood products to prevent transfusion-associated graft-versus-host disease. Patients with HIV do not appear to be at risk for this condition and can receive nonirradiated blood. __________

A 22-Year-Old Woman Is Evaluated for Fever, Diarrhea, and Skin