ABSTRACT
Pulmonary alveolar microlithiasis (PAM) is a rare entity, presenting mostly as an incidental finding. This disease has an autosomal recessive inheritance with inactivating mutations in the gene "solute carrier family 34 member 2". The present study was conducted to bring attention to this rare though preventable disease. The study was a cross-sectional descriptive study, conducted at the Department of Pathology, of a tertiary care hospital in New Dehli-India. PAMs were incidentally seen in two patients diagnosed with micronodular hepatic cirrhosis leading to reanalysis of 212 autopsies, retrospectively. Statistical analysis was done using Stata 14.0. We observed three forms (Type A, B and C) of round hyaline bodies measuring in diameter with thin delicate, radiating fibrils. These bodies were PAS positive, showed black discolouration of the pigment with von Kossa stain and birefringence on polarized microscopy using Congo red stain, however the refringence was light green as compared to apple green birefringence seen with amyloid deposition. PAM has a slow progressive course leading to a high rate of incidental detection. Drugs known to inhibit the micro-crystal growth of hydroxyapatite may slow the disease progression. The family members of patients with PAM may also be kept on follow up with regular imaging. Key messages: It is important to bring out the incidental finding as, seemingly innocuous observations may provide valuable insight into incurable diseases, especially rare diseases.
Subject(s)
Humans , Male , Adult , Middle Aged , Incidental Findings , Lung Diseases/pathology , Autopsy , Calcification, Physiologic , Rare DiseasesSubject(s)
Diagnosis, Differential , Humans , Male , Middle Aged , Molluscum Contagiosum/blood , Molluscum Contagiosum/complications , Molluscum Contagiosum/diagnosis , Recurrence , T-Lymphocytopenia, Idiopathic CD4-Positive/blood , T-Lymphocytopenia, Idiopathic CD4-Positive/complications , T-Lymphocytopenia, Idiopathic CD4-Positive/diagnosisABSTRACT
Thirty years female underwent allogenic peripheral blood stem cell transplantation for chronic myeloid leukaemia--chronic phase. She developed grade II acute skin graft-versus-host disease (GVHD) which was treated with cyclosporine and a short course of steroids. She developed extensive chronic GVHD of the skin and liver three hundred days post-transplant. She was managed with the standard immunosuppressants with partial response of liver dysfunction but no response of skin lesions. She showed a good response to therapy with resolution of skin lesions after treatment with thalidomide.
Subject(s)
Adult , Female , Graft vs Host Disease/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/surgery , Peripheral Blood Stem Cell Transplantation/adverse effects , Skin Diseases/drug therapy , Thalidomide/therapeutic useABSTRACT
Polymorphous Low Grade Adenocarcinoma (PLGA) arises characteristically in the minor salivary glands. Histological diagnosis of this tumour has improved following awareness and well characterized histologic features. However cytologic diagnosis is difficult. Ten cases of PLGA were identified on review of 61 cases of palatal salivary gland tumours aspirated over a 17 year period. Seven of these had been diagnosed as salivary gland tumours but had not been further characterized, while two cases had been diagnosed as adenoid cystic carcinoma initially. In only one case a differential diagnosis between pleomorphic adenoma and PLGA had been considered. Cytologic diagnosis of PLGA is difficult but possible if an attempt is made to identify specific cytologic criteria.