Nutcracker Syndrome with Thin Basement Membrane Disease: A Case Report / 대한내과학회지

Nutcracker syndrome is caused by external compression of the left renal vein and is characterized by various symptoms, such as hematuria and left flank pain. However, long-standing gross hematuria is uncommon. We report the case of a 23-year-old woman who had had painless gross hematuria for several months. Kidney biopsy revealed thin basement membrane disease, but this did not explain the gross hematuria with proteinuria. Renal venography and Doppler ultrasonography showed increased diameter and velocity ratios between the left renal vein and inferior vena cava. Cystoscopy revealed that the hematuria originated from the left ureteral orifice. These results indicate coexisting Nutcracker syndrome. Nutcracker syndrome with thin basement membrane disease should be considered in the differential diagnosis of a patient who has long-standing gross hematuria.

Refractory Pleural Effusion in Systemic Lupus Erythematosus Treated by Pleurectomy

Pleural effusion is a common pulmonary manifestation of systemic lupus erythematosus (SLE) and often occurs as bilateral exudative pleural effusion. The condition usually responds quickly to corticosteroid therapy. However, massive pleural effusion refractory to immunosuppressive drugs has rarely been reported; thus, the proper therapeutic modality is largely decided on a case-by-case basis. In this case, we describe successful treatment with surgical pleurectomy for massive refractory pleural effusion in a patient with SLE.

A Fulminant Case of Clostridium perfringens Septic Shock caused by a Liver Abscess with Massive Hemolytic Anemia / 대한내과학회지

A 65-year-old female visited the emergency room for severe back pain radiating to the neck. Aortic dissection computed tomography revealed a ruptured liver abscess and large pneumoperitoneum. Although emergent percutaneous drainage of the liver abscess and aggressive resuscitation were performed, massive hemolytic anemia and disseminated intravascular hemolysis developed and she subsequently died, 11 hours after her visit to the emergency room. Clostridium perfringens was identified in a blood culture obtained at the emergency room. We report this case because refractory septic shock due to a liver abscess and massive intravascular hemolytic anemia caused by Clostridium perfringens in a healthy female is rare.

Hemophagocytic Lymphohistiocytosis Occurring after Induction Chemotherapy in Acute Myelocytic Leukemia / 대한내과학회지

Hemophagocytic lymphohistiocytosis (HLH), associated with acute myelocytic leukemia (AML), is a very rare disease. We here report a case of HLH occurring after induction chemotherapy for AML. AML-associated HLH can be triggered by AML itself, by chemotherapeutic agents, or by infectious complications. Our patient developed a high-grade fever of unknown cause, bilateral pulmonary infiltrates, and shock after successful treatment of AML with induction chemotherapy, and had high serum ferritin, hypertriglyceridemia, hypofibrinogenemia, hemophagocytic histiocytes in bone marrow, low natural killer cell activity, and elevated soluble interleukin-2 receptor levels. A diagnosis of HLH was made. Dexamethasone, cyclosporine, and etoposide were given and allogeneic hematopoietic stem cell transplantation was performed. Careful suspicion of HLH may be warranted if a patient experiences fever of unknown etiology, high ferritin levels, and liver dysfunction during AML treatment.