A 32-year-old man comes to the emergency department due to a persistent nosebleed lasting an hour. Over the past week, he has had several episodes of epistaxis that stopped after prolonged nose pinching. The patient has also noticed more fatigue and skin bruising than usual over the past month. He has not had any recent nasal trauma or upper respiratory tract infections and does not use any intranasal medications. The patient does not use tobacco or alcohol and maintains a vegan diet. Temperature is 37.2 C (99 F), blood pressure is 130/80 mm Hg, pulse is 96/min, and respirations are 18/min. Physical examination shows dried blood in the right nostril with no intranasal lesions. No enlarged lymph nodes are present. Lung fields are clear to auscultation and heart sounds are normal. The abdomen is soft and nontender with no hepatosplenomegaly. Scattered ecchymoses are present on the extremities. Laboratory results are as follows: Which of the following is most likely associated with this patient's current condition?

Question

A 32-year-old man comes to the emergency department due to a persistent nosebleed lasting an hour.  Over the past week, he has had several episodes of epistaxis that stopped after prolonged nose pinching.  The patient has also noticed more fatigue and skin bruising than usual over the past month.  He has not had any recent nasal trauma or upper respiratory tract infections and does not use any intranasal medications.  The patient does not use tobacco or alcohol and maintains a vegan diet.  Temperature is 37.2 C (99 F), blood pressure is 130/80 mm Hg, pulse is 96/min, and respirations are 18/min.  Physical examination shows dried blood in the right nostril with no intranasal lesions.  No enlarged lymph nodes are present.  Lung fields are clear to auscultation and heart sounds are normal.  The abdomen is soft and nontender with no hepatosplenomegaly.  Scattered ecchymoses are present on the extremities.  Laboratory results are as follows:  Which of the following is most likely associated with this patient's current condition?

Quizplus · Accepted Answer

11ec25c8_d408_dbea_bc81_9b03d296c10f_MD0003_00 This patient with fatigue, prolonged bleeding, and easy bruising has pancytopenia, coagulopathy, and elevated lactate dehydrogenase, strongly suggesting acute promyelocytic leukemia (APML), a form of acute myeloid leukemia (AML). AML is a clonal myeloproliferative disorder characterized by the accumulation of blastic or immature myeloid cells in the bone marrow and peripheral blood. Patients often develop fatigue and symptoms related to >1 cytopenias, including fatigue/weakness (anemia), bleeding/bruising (thrombocytopenia), and/or infection (granulocytopenia). Examination may show pallor and ecchymosis, but lymphadenopathy and hepatosplenomegaly are rare. Leukocyte count may be elevated (sometimes >100,000/mm³), normal, or low. > One unique type of AML is APML, which is characterized by life-threatening coagulopathy due to disseminated intravascular coagulation (prolonged PT/active PTT, hypofibrinogenemia). In APML, bone marrow biopsy would reveal atypical promyelocytes. (Choice B) Von Willebrand disease, an inherited bleeding disorder associated with factor VIII deficiency, is often asymptomatic but may present with mucocutaneous bleeding and easy bruising. Platelets are usually normal or mildly (not severely) low, pancytopenia is uncommon, and coagulation studies other than activated PTT should be normal. (Choice C) Vitamin B₁₂ is derived from meat ingestion; therefore, patients on a vegan diet are at increased risk of deficiency. Deficiency can cause pancytopenia, but coagulation studies should be normal. (Choice D) Malignancies that cause blastic bone lesions may infiltrate the bone marrow, resulting in pancytopenia. Most patients with diffuse blastic lesions have bone pain. In addition, coagulopathy is atypical. (Choice E) Hodgkin lymphoma may cause a mediastinal mass, pancytopenia, and an elevated lactate dehydrogenase level. However, B symptoms (eg, fever, night sweats) and painless lymphadenopathy are usually prominent (unlike in this patient). (Choice F) Multiple myeloma is associated with monoclonal protein on electrophoresis and may sometimes cause pancytopenia (isolated anemia is most common). However, disseminated intravascular coagulation would be unusual, and patients typically have bone pain. Educational objective: Acute myeloid leukemia (AML) is the most common acute leukemia in adults and is typically associated with fatigue and symptoms related to >1 cytopenias. Acute promyelocytic leukemia is a unique form of AML that often presents with life-threatening coagulopathy due to disseminated intravascular coagulation. > References: Significance of fibrinogen, D-dimer, and LDH levels in predicting the risk of bleeding in patients with acute promyelocytic leukemia. (http://www.ncbi.nlm.nih.gov/pubmed/20554322)

A 32-Year-Old Man Comes to the Emergency Department Due to a Persistent