Acute promyelocytic leukemia (APL) with an unusual cytogenetic presentation.
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A 43-year-old woman was admitted with progressive leg pains and weakness and was found to have rhabdomyolysis. Prior to this admission the patient had hypercalcemia, but this returned to normal following treatment with calcitonin. During the hospitalization, she developed the syndrome of calciphylaxis consisting of necrotic skin and muscle associated with vascular calcification. This is the first case report of rhabdomyolysis caused by calciphylaxis in a patient without chronic renal failure.
The anatomy of a black eye is examined in a series of cadaver dissections in which a previously unreported fascial system of the lower eyelid is identified. This fascia originates at the orbital rim, and is in continuity with the orbital septum and with the periosteum of the orbital floor and anterior maxillary wall. This fascia contributes to the thickened area along the orbital rim called the arcus marginale. At the level of the orbicularis oculi muscle, this fascia was noted microscopically to fuse with a fibrous septa of the superficial cheek fat. This creates one long continuous membrane from the orbital rim above to the cheek skin below. Dye injection techniques show that this membrane is impermeable and traps injected dye in the same place where a black eye forms. After periorbital injury, extravasated hemoglobin pigment is confined to the area above the cutaneous insertion of this membrane. This fascial system has been named the septum malaris: malar describes its origin along the orbital rim of the cheek, and septum further describes the partitioning nature of this ultra-thin membrane.
An 18-year-old Caucasian woman has been followed since age 12 years for Ehlers-Danlos syndrome (EDS) with easy bruisability and "cigarette paper scars." Her chief complaint at age 17 years was tooth mobility, especially in the anterior mandible, necessitating the removal of the four incisors. Initial biochemical analysis of cultured skin fibroblasts indicated the presence of pepsin-sensitive type III collagen. Subsequent analysis of cultured skin fibroblasts by the same laboratory and another laboratory found no abnormality in the type III collagen with or without protease treatment. This is in distinction to the finding of abnormal type III collagen in the only two reported patients with EDS and early-onset periodontitis who have had collagen analyses. One of them was diagnosed as EDS type IV and the other as EDS type VIII, although the defects of type III collagen were consistent with EDS type IV. The defect in type III collagen in some patients with early periodontitis and the considerable overlap of the clinical manifestations of EDS types IV and VIII point out the need for further studies of collagen formation and maturation in any patient who has early periodontitis and who has been classified with EDS type IV or VIII.
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Red cells of 3 patients with the painful bruising syndrome showed morphological abnormalities. In the fraction not sedimenting in Ficoll/Isopaque gradient centrifugation, some of the cells had club-shaped processes, mitochondria, nuclear remnants, and vacuoles. In freeze-etch preparations, 90% of the red cells showed membrane elevations at pH 7.4 and 25% at pH 6.4, while in freeze-etch preparations of controls these values were 55 and 0 respectively. In addition, rouleaux formation was markedly enhanced in the preparations of blood of patients with the painful bruising syndrome.
Use of Reston foam along with a pressure garment after liposuction results in three salutary effects: less waviness in the skin surface, a more comfortable recovery, and less blood loss and ecchymoses. Free fat cells are thought to be redistributed evenly by the pressure of the foam plus the pressure garment. The result is a smoother skin surface with less waviness. Reston foam is also comfortable under the pressure garment and there is less immediate postoperative tenderness. In addition, the use of the foam, in conjunction with the pressure garment, results in less postoperative bleeding and bruising. Consequently, there is also a decrease in the amount of postoperative fall in hematocrit. Since there is less of a drop in hematocrit and hemoglobin, there is also less postoperative anemia. The mechanism involved in the reduction in blood loss is thought to be a combination of overcoming capillary pressure and stabilization of the skin and subsurface blood vessels.
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An acquired factor VIII inhibitor was found in an 82-year-old woman who presented with numerous spontaneously appearing ecchymoses. Coagulation studies revealed the presence of a prolonged activated partial thromboplastin time that was not corrected by 1:1 mixture with normal fresh plasma after a 2-h incubation. Factor VIII:C was 4%, and the titer for factor VIII inhibitor was 9 Bethesda units. Three months later, after a retroperitoneal hemorrhage, a lymphocytosis was found in her peripheral blood with morphological and surface immunophenotype characteristics of B-cell chronic lymphocytic leukemia, later confirmed by bone marrow morphological and immunocytochemical examinations. To our knowledge, this is the first report of an autoimmune factor VIII inhibitor associated with chronic lymphocytic leukemia.
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14 fresh cadavers of adult persons were placed with the head or body tilted in atypical positions 3 to 65 hours after death. The persons concerned were neither asphyxiated nor had they suffered cranial trauma. Hypostasis in the head and neck occurred more rapidly and intensively the deeper the cranial parts of the body were placed. In the simulated cadaver positions, artificial ecchymoses were observed in only a few places, mainly in the eyelids, the conjunctiva, the galea and the temporal muscles except when the head was vertically downwards. Just as with the postmortem haematomas, the hypostatic settling to the plane of the heart was decisive.
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In our study of six patients who underwent fat aspiration with a syringe on one side and with a pump on the other, there was notably less blood in the fat aspirate from the syringe and less bruising and morbidity in the postoperative period. The only variable in the study for each patient was the method of aspiration.
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A 54-year old anorectic patient with painful bruising syndrome (Gardner-Diamond syndrome) suffered from various gastrointestinal and psychologic complaints. The episodes of painful bruising could be provoked following intradermal injection of washed red blood cells within 96 h. The pathogenesis of this rare entity is more likely due to a psychiatric alteration rather than to the immunological mechanisms, as had been postulated two decades ago. Nevertheless, the Gardner-Diamond-syndrome should be included in the differential diagnosis of ecchymotic bleeding.