A 53-year-old woman recently diagnosed with systemic sclerosis comes to the emergency department with headaches, increasing dyspnea, malaise, and nausea. Her medications include lansoprazole for heartburn and nifedipine for finger discoloration and pain. Physical examination reveals a distressed and anxious woman. Her blood pressure is 235/117 mm Hg and pulse is 120/min. She has visible skin tightening over the face, forearms, chest, and legs. She has difficulty opening her mouth due to tightening of the skin around the mouth. Lung examination shows bibasilar inspiratory crackles. Laboratory studies are as follows: Which of the following red blood cell abnormalities is most likely to be present in the peripheral blood smear?

Question

A 53-year-old woman recently diagnosed with systemic sclerosis comes to the emergency department with headaches, increasing dyspnea, malaise, and nausea.  Her medications include lansoprazole for heartburn and nifedipine for finger discoloration and pain.  Physical examination reveals a distressed and anxious woman.  Her blood pressure is 235/117 mm Hg and pulse is 120/min.  She has visible skin tightening over the face, forearms, chest, and legs.  She has difficulty opening her mouth due to tightening of the skin around the mouth.  Lung examination shows bibasilar inspiratory crackles.  Laboratory studies are as follows:  Which of the following red blood cell abnormalities is most likely to be present in the peripheral blood smear?

Quizplus · Accepted Answer

This patient's marked hypertension and acute kidney injury in the setting of systemic sclerosis (scleroderma) suggests scleroderma renal crisis (SRC), which can occur in up to 20% of patients with diffuse cutaneous systemic sclerosis (SSc). SRC usually occurs within the first 5 years of SSc diagnosis. The likely mechanism involves increased vascular permeability, activation of the coagulation cascade, and increased renin secretion. Patients typically develop sudden onset of renal failure (without previous kidney disease), and malignant hypertension (eg, headache, blurry vision, nausea). Urinalysis can be normal or show mild proteinuria. Peripheral blood smear can show microangiopathic hemolytic anemia (similar to hemolytic uremic syndrome/thrombotic thrombocytopenic purpura) or disseminated intravascular coagulation with fragmented red blood cells (eg, schistocytes) and thrombocytopenia. schistocytes (Choice A) Burr cells (also known as echinocytes) are spiculated appearing red blood cells (RBCs) with serrated edges that can be seen in liver disease and end-stage renal disease (ESRD). However, burr cells are less common in the acute kidney injury seen in SRC. Burr cells (Choice B) Howell-Jolly (HJ) bodies are basophilic remnants of the nucleus that appear as small, black pellets in RBCs. They are seen in patients with a history of splenectomy or functional asplenia. HJ bodies are not seen in healthy individuals as a normal spleen efficiently removes them. Howell-Jolly (HJ) bodies (Choice D) Spur cells (acanthocytes) are RBCs with irregularly sized and spaced projections that are most commonly seen in liver disease. Spur cells (Choice E) Target cells are red blood cells with a central density surrounded by pallor (bull's eye appearance). They are usually seen in patients with hemoglobinopathies (eg, thalassemia) or chronic liver disease (especially obstructive liver disease). Target cells Educational objective: Scleroderma renal crisis typically presents with acute renal failure (without previous kidney disease) and malignant hypertension (eg, headache, blurry vision, nausea). Urinalysis may show mild proteinuria. Peripheral blood smear can show microangiopathic hemolytic anemia with fragmented red blood cells (eg, schistocytes) and thrombocytopenia. References: Scleroderma renal crisis. (http://www.ncbi.nlm.nih.gov/pubmed/24833760) Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. (http://www.ncbi.nlm.nih.gov/pubmed/20012923)

A 53-Year-Old Woman Recently Diagnosed with Systemic Sclerosis Comes to the Emergency