Intracranial chloroma may occur in leukemia, although they are rare. A 23-year-old female complained diplopia. Brain magnetic resonance MR imaging showed tumors in the both cavernous sinus , both tentorial and anterior falx. Gamma-Knife radiosurgery was performed with maximal dose; 20Gy, marginal dose; 10Gy. Peripheral blood smear revealed leukemia, and bone marrow aspiration biopsy showed acute lymphocytic leukemia. Two weeks later, MR image for the stereotactic biopsy noticed markedly decreased tumor size. Biopsy result was lymphocytic leukemia. She received conventional radiation therapy, chemotherapy, and bone marrow transplantation. Brain involvement by acute lymphocytic leukemia is very rare. Even though chloroma are sensitive to radiation therapy, prognosis is poor because of the gravity of the underlying disease and association with impending blast transformation. The authors reports a intracranial chloroma by acute lymphocytic leukemia.
Biopsy ; Biopsy, Needle ; Bone Marrow ; Bone Marrow Transplantation ; Brain ; Cavernous Sinus ; Diplopia ; Drug Therapy ; Female ; Gravitation ; Humans ; Leukemia ; Leukemia, Lymphoid* ; Lymphocyte Activation ; Magnetic Resonance Imaging ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Radiosurgery ; Sarcoma, Myeloid ; Young Adult

Lymphocytic interstitial pneumonia (LIP) is a benign lymphoproliferative disorder characterized histologically by diffuse infiltration with predominantly mature lymphocytes, plasma cells and reticuloendothelial cells in the alveolar septae and along the lymphatic vessels. LIP is an uncommon disease frequently associated with autoimmune diseases such as Sj?gren's syndrome, systemic lupus erythematosus, chronic active hepatitis, thyroiditis, myasthenia gravis, autoimmune hemolytic anemia and Human Immunodeficiency Virus (HIV) or Epstein- Barr virus (EBV) infection. LIP can progress to pulmonary or systemic lymphoma. LIP in children has variable disease courses such as spontaneous resolution, episodic worsening or progression to frank respiratory failure. Intravenous immunoglobulin and corticosteroids yield variable results, and some children may respond to them for several years. We report herein a case of EBV-associated, HIV-negative LIP diagnosed by thoracoscopic lung biopsy and a clonality study in a 5 year-old boy who was admitted with recurrent coughing and dyspnea. The patient was treated and fully recovered with methylprednisolone and high dose acyclovir. At the 3-year follow-up, he showed no clinical symptom despite increased EBV copy in bronchoalveolar lavage and progressive bronchiectasis in the right middle lobe.
Acyclovir ; Adrenal Cortex Hormones ; Anemia, Hemolytic, Autoimmune ; Autoimmune Diseases ; Biopsy ; Bronchiectasis ; Bronchoalveolar Lavage ; Child ; Coat Protein Complex I ; Cough ; Dyspnea ; Follow-Up Studies ; Hepatitis, Chronic ; Herpesvirus 4, Human ; HIV ; Humans ; Immunoglobulins ; Lip ; Lung ; Lung Diseases, Interstitial ; Lupus Erythematosus, Systemic ; Lymphatic Vessels ; Lymphocytes ; Lymphoma ; Lymphoproliferative Disorders ; Methylprednisolone ; Myasthenia Gravis ; Plasma Cells ; Respiratory Insufficiency ; Thyroid Gland ; Thyroiditis ; Viruses

Background/Aims: Although pediatric ulcerative colitis (UC) has a different phenotype and clinical course than adult UC, its clinical features and outcomes are poorly defined, especially in Asian populations. This study investigated the clinical features and long-term outcomes of pediatric UC in a Korean population. Methods: We retrospectively analyzed 208 patients aged <18 years diagnosed with UC between 1987 and 2013. The patient characteristics at diagnosis according to the Paris classification and the clinical course were analyzed. Results: The male-to-female ratio was 1.3:1, and the median patient age was 15.5 years. At diagnosis, 28.8% of patients had proctitis (E1), 27.8%, left-sided colitis (E2); 5.2%, extensive colitis (E3); and 38.2%, pancolitis (E4). The cumulative probabilities of extension after 5, 10, 15, and 20 years were 32.7%, 40.4%, 52.5%, and 65.8%, respectively. Eighteen patients underwent colectomy, and three patients had colorectal cancer. The cumulative probabilities of colectomy after 5, 10, 15, and 20 years were 7.1%, 8.9%, 12.6%, and 15.6%, and those of colorectal cancer after 10, 15, and 20 years were 0%, 2.1%, and 12.0%, respectively. The disease extent, Pediatric Ulcerative Colitis Activity Index severity, and systemic corticosteroid therapy were significant risk factors for colectomy. The development of primary sclerosing cholangitis was significantly associated with colorectal cancer. Conclusions This study provides detailed information on the disease phenotype and long-term clinical outcomes in a large cohort of Korean children with UC. They have extensive disease at diagnosis, a high rate of disease extension, and a low rate of cumulative colectomy.