Primary adult-onset macrophage activation syndrome with multisystemic tissue phagocytosis

The histiocyte disorders are divided into the following 3 categories according to the specific lineage of the histiocytes involved and their biological behavior: the dendritic cell-related disorders, which include Langerhans cell histiocytosis and dermal dendrocyte disorders; the macrophage cell disorders, hemophagocytic lymphohistiocytosis being the main entity; and the malignant histiocyte disorders. We present a case of a 36-year-old woman who was referred to our hospital because of fever of unknown origin, lethargy, anemia, and impaired hepatic function. Following a thorough investigation, we diagnosed extensive histiocyte-mediated phagocytosis in many areas (skin, liver, bone marrow), without any identifiable cause. The disease was controlled by immunosuppressive therapy, and the patient remains in complete remission. This case supports the concept of idiopathic generalized histiocyte activation as a distinct entity; this putative disease entity produces massive phagocytosis, regardless of the type of histiocyte differentiation. Similar cases necessitate further study for classification and management

Spontaneous rupture of a giant renal angiomyolipoma—Wunderlich’s syndrome: Report of a case

AbstractIntroductionHerein we present a rare case of pontaneous rupture of a giant renal angiomyolipoma (AML), with symptoms of hypovolemic shock (Wunderlich’s syndrome), which was managed by urgent total nephrectomy.Presentation of caseA 53 year old female was transferred to the emergency room with progressive acute painful swelling of the left lateral abdominal area, duration of 5h. An emergent ultrasonic examination, revealed a heterogeneous—solid mass with maximum diameter of 23cm, with probable origin from the left kidney. Due to worsening of the clinical status (hypovolemic shock), loss of consciousness and acute drop of haematocrit level to 17.8%, the patient was urgently intubated in the emergency room and transferred to the operating theater. A giant haemorrhagic mass was found originating from the left kidney, which removed en-block with the left kidney. The patient was transferred to the intensive care unit. Her recovery was uneventful. The histopathologic examination revealed a giant renal angiomyolipoma (25×18×8cm) with extensive bleeding.DiscussionEnlarged renal AMLs can rupture. This can be sudden and painful with manifestations of hypovolemic shock. The management of AMLs has been correlated with symptoms. Patients with life-threatening retroperitoneal haemorrhage, require urgent exploration as retroperitoneal bleeding can lead to severe complications, increasing morbidity.ConclusionIn case of giant angiomyolipoma with intratumoral haemorrhage, and symptoms of Wunderlich’s syndrome, partial or total nephrectomy is a good treatment option in order to save the patient’s life