Langerhans cell histiocytosis of the skull on cytologic squash preparations

We present a case in which a primary cytodiagnosis of Langerhans cell histiocytosis (LCH) of the skull was made using squash preparations. The patient, a 25-year-old male, presented with raised intracranial pressure and decreased visual acuity. Magnetic resonance imaging revealed a large skull lesion with osteolytic features in the left frontal bone. The patient underwent surgical resection by the extended basal frontal epidural approach. The squash preparation smears were cellular and demonstrated a mixed population of small, mature lymphocytes, eosinophils, and a high histiocytes content. The histiocytes occurred as isolated or loosely cohesive and clustered. They possessed abundant cytoplasm with rounded cell shape and had characteristic nuclear features, composed of fine chromatin and delicate nuclear membranes. The cytologic features of these histiocytes were consistent with Langerhans cells (LCs). A final impression of LCH of the skull was rendered. Subsequent histopathology confirmed the diagnosis. LCs reacted with both S-100 protein and CD1a immunohistochemically. The demonstration of Birbeck granules on electron microscopic study was also noted. Whenever squash preparation yields a mixed population of mature lymphocytes, eosinophils, and histiocytes, the cytologists should be aware of and consider LCH as a diagnostic possibility.     

Feasibility of a percutaneous technique for repairing proximal femora with simulated metastatic lesions

Fracture of the proximal femur due to metastatic disease is a significant cause of morbidity and mortality among breast cancer patients. Prophylactic surgical fixation is advised for patients at risk of fracture and typically involves placement of an orthopaedic implant. We propose that some proximal femora with metastases can be repaired by removing the lesion and filling the resulting defect with bone cement (polymethylmethacrylate), a procedure that could be performed percutaneously without the use of hardware. We studied the strengths of 12 matched pairs of cadaveric proximal femora under single-limb stance loading. One femur from each pair remained intact, while a simulated metastatic lesion, measuring approximately 75% of the neck diameter, was burred into the neck of the contralateral femur. The defects were repaired using a procedure similar to the one proposed. Femoral strength was measured via mechanical testing to failure. The strengths of the repaired femora averaged 94.7% of the strength of their respective contralateral intact femur (standard deviation, 8.7%). These findings suggest that the proposed procedure may be useful for some patients with metastases in the femoral neck. If the proximal femur could be safely repaired using the proposed technique in place of conventional surgical fixation, the patient would benefit from a shorter and less invasive surgical procedure, less pain and discomfort, greatly reduced recovery time, and a shorter hospital stay-all at a much lower cost.     

Vitamin D receptor polymorphism among rickets children in Mongolia

BACKGROUND: It was reported that 32% of children under five years old in Mongolia had symptoms of rickets. Vitamin D receptor (VDR) gene polymorphism has received attention in relation to bone metabolism. We therefore investigated whether VDR polymorphism is related to high prevalence of rickets in Mongolia and to bone properties in childhood. METHODS: We conducted a case-control study in Ulaanbaatar involving 80 children aged 7-10 years with a history of rickets (cases) and 72 children with no history of rickets (controls). VDR polymorphism was assessed using BsmI, ApaI, and TaqI, and bone properties were determined by measuring age standardized tibial cortical speed of sound (TCSOS). FINDINGS: Each allelic frequency was verified to satisfy the Hardy-Weinberg equilibrium in cases, controls, and the total sample. The VDR polymorphisms among cases (BB 3%, Bb 18%, bb 80%; AA 15%, Aa 38%, aa 47%; and TT 81%, Tt 17%, tt 3%) did not differ significantly from those among controls (BB 1%, Bb 13%, bb 86%; AA 16%, Aa 46%, aa 38%; and TT 86%, Tt 13%, tt 1%). There were no significant differences in TCSOS according to the VDR genotype among either cases or controls. CONCLUSIONS: The VDR polymorphism does not play a major role in the development of rickets in Mongolia and has no effect on TCSOS in childhood.     

A case of nephrotic syndrome due to lupus nephritis which was controlled with low-density lipoprotein apheresis.

Our report discusses a 29 year old female patient with nephrotic syndrome due to lupus nephritis, biopsy-proven World Health Organization classification Types IVb and V that was controlled with low-density lipoprotein (LDL) apheresis. She was initially treated with steroid therapy, including methylprednisolone pulse therapy, and the serological activity of her systemic lupus erythematosus was suppressed. However, her nephrotic state, accompanied by severe hyperlipidemia, persisted despite the steroid therapy. Since we could not obtain her consent to administer immunosuppressants such as cyclophosphamide, we tried to treat her using LDL apheresis (LDL-A). We found that her urine protein excretion, hyperlipidemia, hypoalbuminemia, and renal function improved following the initiation of LDL-A. This suggests that LDL-A may be an effective therapy for nephrotic syndrome due to lupus nephritis through short-term amelioration of hyperlipidemia.     

PANDEMIC OF NEW TYPE OF CONJUNCTIVITIS

A new type of conjunctivitis was prevailing in a pandemic fashion all over South-East Asia and Japan in 1970 and 1971. The disease is characterised by severe subconjunctival hæmorrhage, but its clinical course is usually benign and complete recovery comes within a fortnight. A cytopathic agent has been isolated by primary human embryonic kidney cell culture of conjunctival scrapings or swabs taken from patients with acute hæmorrhagic conjunctivitis (A.H.C.) in three different outbreaks in Japan. One of the earliest isolates which was chosen for detailed study showed general characteristics of enterovirus, but it was not neutralised by intersecting pools of antisera against known enteroviruses, and it was not pathogenic to suckling mice. It is suggested that the agent is an enterovirus of a new serotype. 10 pairs of acute and convalescent sera were tested, and there were definite neutralising antibody rises against this agent in all of them. No specific bacteria were found from cultures of conjunctival swabs. It is concluded that A.H.C. in Japan is caused by a hitherto unknown type of enterovirus and that outbreaks in other countries may be due to a similar agent.     

Protein-losing enteropathy caused by mesenteric venous thrombosis with protein C deficiency

A 64-year-old man presented with leg edema and hypoproteinemia. His alpha-1 antitrypsin clearance rate of 174 mL/day indicated the presence of protein-losing enteropathy (PLE). Computed tomographic scans demonstrated thickened ileal wall and mesenteric edema. Angiography revealed occlusion in a peripheral branch of the superior mesenteric vein. Furthermore, both the patient and his son had low protein C levels. The patient was treated successfully with partial resection of the ileum. Histologic examination of the resected ileum revealed multiple erosions and submucosal fibrosis with organized venous thrombi in the mesenteric veins. This is the first case report of PLE caused by mesenteric venous thrombosis, and our findings suggest that serum protein was lost through erosion of the ileum caused by ischemia due to mesenteric venous thrombosis.