Bone Marrow Infiltration in Rosai-Dorfman Disease.

Rosai-Dorfman disease (RDD) is a rare and benign nonLangerhans cell histiocytosis. RDD commonly affects children and young adults typically presenting with massive, painless, and bilateral cervical lymphadenopathy. Extranodal involvement is present in about 40% of cases, and bone marrow infiltration is rare and unusual. RDD prognosis is usually good, however, involvement of bone marrow is associated with poor prognosis. We report a case of RDD with bone marrow involvement occurring in a 5-year-old female. She was admitted for asthenia, gingival bleeding, and diffuse bone pain without fever. Physical examination showed pallor, petechiae over the abdomen, painless and bilateral cervical lymphadenopathy, and several bony nodules, without splenomegaly or hepatomegaly. Cell blood count revealed microcytic hypochromic anemia with thrombocytopenia. Bone marrow aspiration showed infiltration by large histiocytes with hypochromatic nuclei and abundant vacuolated cytoplasm without emperipolesis. Biopsies of bone marrow, lymph node, and bone revealed immunohistochemical features of RDD: the histiocytes were positive for CD68 and S100 protein, but negative for CD1a. The patient initially received symptomatic treatment. One week after admission, she died of septic shock before the final establishment of RDD diagnosis. This case report highlights that bone marrow involvement in RDD is rare and associated with poor prognosis. We also aim to emphasize the obligation of bone marrow exploration in patients with RDD and presenting cytopenias in order to make an early diagnosis of bone marrow infiltration.

Protein-Losing Enteropathy Associated with Thrombotic Microangiopathic Features Revealing Hirschsprung's Disease in a Child.

We report the case of a 4-year-old boy with protein-losing enteropathy, leading to severe malnutrition. Associated thrombotic microangiopathic features made diagnosis difficult and challenging.

In vitro correlation of the level of inferior alveolar canal with CBCT imaging.

PURPOSE: The aim was to correlate the level of the inferior alveolar canal on cadaver specimens and as evaluated with three-dimensional radiographic cone-beam computed tomography (CBCT) imaging. METHODS: Twenty-one skulls with intact mandibles (42 inferior alveolar canals) were imaged with an I-CAT Cone-Beam 3D Imaging System and multi-planar reformatted images obtained were scored to assess the level of the canal (Level I, Level II, and Level III). The mandibles were later dissected and level of the canal was directly visualized, scored, and further correlated with results from CBCT imaging. RESULTS: There was no difference in the level of the inferior alveolar canal between the dissected cadaver specimen and CBCT images. Also, there was no statistically significant difference in the level of the canal among the gender assigned to the specimens and corresponding CBCT images. There were no statistical differences in the level of the canal (cadaver specimen vs. CBCT) between the left and right sides of the specimens, as studied individually. CONCLUSION: Our study indicates that there is close correlation of the level of the inferior alveolar canal between cadaver specimens and CBCT images. Well-designed studies would be required to further validate the findings in this study.