Cirrhosis is a known cause of thrombocytopenia but it is important to consider other etiologies when the degree of thrombocytopenia is severe, especially in light of impending life-threatening bleeding. One must always maintain a low threshold for additional diagnostic entities when patients present acutely and confirmatory testing reveals profound thrombocytopenia. A 34-year-old man with cirrhosis secondary to Hepatitis C and alcohol abuse presented with persistent bleeding from preexisting oral ulcers and hematuria. Patient denied melena, hematemesis or hematochezia. His past medical history was significant for pancytopenia secondary to cirrhosis, active hepatitis C infection and hypersplenism. He denied any recent change in his medications nor taking any herbal medications or supplements. Vital signs were normal on admission. Physical examination was positive for dried blood on the lips and hepatosplenomegaly. Lab work revealed a platelet count of 0 with chronic leukopenia and anemia. His baseline platelet count is approximately 35,000. Urine analysis indicated gross blood. Coagulation workup was not suggestive of Disseminated Intravascular Coagulation (DIC). Peripheral smear was significant for complete lack of platelets without schistocytes. He was started on daily platelet transfusions with minimal change in his platelet count. A diagnosis of secondary Immune Thrombocytopenic Purpura (ITP) was made and therapy was initiated with intravenous immunoglobulin (IVIG) and dexamethasone. His platelet count failed to improve with worsening hematuria. He also received Rituximab, Romiplostim infusions and high dose methylprednisolone. The patient underwent splenic artery embolization three times. In spite of all efforts he continued to have hematuria and bleeding from intravenous lines with only transient rise in counts. He was taken for laparoscopic splenectomy with a platelet count of 35,000; following which the bleeding subsided and his platelet count improved to 100,000. Patient had a complicated hospital course but was eventually discharged home and currently his platelet counts are within normal limits. This patient appeared to have developed secondary ITP from his active Hepatitis C. Though he had chronic thrombocytopenia from cirrhosis and splenomegaly, it would be unusual to see this degree of platelet drop from these causes alone. ITP is a diagnosis of exclusion and bleeding is usually not proportionate to level of thrombocytopenia as in this patient. This case illustrates the fact that a clinician must have a low threshold for expanding the differential diagnosis of thrombocytopenia, especially diagnoses that are likely to harm the patient such as Thrombotic Thrombocytopenic Purpura, Disseminated Intravascular Coagulation and ITP. This case also demonstrates the challenging nature of managing severe refractory ITP. Splenectomy is the preferred therapy for patients with ITP who are refractory to first-line therapy with glucocorticoids or IVIG and is shown to cause sustained remission in two-thirds of patients.
New Mexico ACP
Mitta, Pradeep R.; Ola M. Azzouqah; Patrick A. Rendon; and Mary E. Lacy. "Clinical vignette: Zero in 60 in 48 hours." (2014). http://digitalrepository.unm.edu/hostpitalmed_pubs/35