CONTENTS The Clue in the Murmur : Post - MI Complications

A 44-year-old quadriplegic woman presented with fevers, hematuria, and a leukemoid reaction of >70,000 cells/mL. Workup for infectious etiologies was negative. A large bladder mass with bilateral lymphadenopathy was discovered by pelvic CT with biopsy revealing a dedifferentiated urothelioma. The cancer was deemed inoperable. The patient was subsequently found to have an elevated GCSF of 182.9 pg/ml (normal range 0.0 – 39.1 pg/ml), consistent with a GCSF paraneoplastic syndrome driving her leukemoid reaction. The patient’s mental status became acutely altered in conjunction with poor lung function, likely consequences of leukostasis. Administration of IV Solumedrol temporarily alleviated these symptoms, enabling her to participate in her end-of-life planning. Unfortunately, the patient developed lung and bladder infections while on steroid therapy and died. IDENTIFICATION / CHIEF COMPLAINT: A 44-year-old quadriplegic woman presented with a 6monther history of intermittent fevers and hematuria. HISTORY: The patient had no reported history of urinary tract infections until approximately 6 months prior to presentation at which time she began experiencing intermittent fevers and hematuria. During this time she had been hospitalized repeatedly for anemia and presumed urosepsis (no outside records available). PHYSICAL ABNORMALITIES: The patient remained communicative and cooperative until hospital day 4, at which time she experienced a sudden decline in her mental and respiratory status. The patient was found to be agitated, disoriented, and though able to follow simple commands was incapable of meaningful communication. Strength, sensation, and reflexes were all unchanged from her baseline disability. LAB RESULTS: Markedly elevated white count, left shifted, mature granulocytes. Elevated GCSF of 182.9 pg/ml (normal range 0.0 – 39.1 pg/ml) DIFFERENTIAL DIAGNOSIS: Leukemoid reactions have been associated with DKA, retroperitoneal hemorrhage, medications (e.g., sulfa, glucocorticoids, GCSF), specific infections (Clostridium difficile, tuberculosis, pertussis), and neoplasms including both sarcomas and carcinomas. DISCUSSION: This report demonstrates the capacity of dedifferentiated urothelial tumors to produce markedly elevated levels of GCSF spurring leukemoid reactions, complicated in this case by steroid-responsive leukostasis. Leukostasis leads to microischemia and hemorrhage in the lungs and brain, resulting in respiratory distress and confusion. Chest x-ray often shows bilateral pleural effusions, as seen in our patient, and there is evidence that steroids reduce leukostasis and improve outcomes even in myelogenous leukemias. It is also worth noting that the differentiation syndrome following ATRA therapy in APML patients is thought by many to be the result of a differentiation-induced leukocytosis with subsequent leukostasis. Interestingly, differentiation syndrome is also treated successfully with dexamethasone therapy.