Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Myeloid, Acute/therapy* ; Mastocytosis/therapy* ; Transplantation Conditioning

Noncardiac chest pain (NCCP) is one of the most common esophageal symptoms and lacks a clearly defined mechanism. The most common cause of NCCP is gastroesophageal reflux disease (GERD). One of the accepted mechanisms of NCCP in a patient without GERD has been altered visceral sensitivity. Mast cells may play a role in visceral hypersensitivity in irritable bowel syndrome. In this case, a patient with NCCP and dysphagia who was unresponsive to proton pump inhibitor treatment had an increased esophageal mast cell infiltration and responded to 14 days of antihistamine and antileukotriene treatment. We suggest that there may be a relationship between esophageal symptoms such as NCCP and esophageal mast cell infiltration.
Adult ; Chest Pain/*etiology ; Esophageal Diseases/*complications/drug therapy ; Esophagus/cytology/pathology ; Female ; Histamine Antagonists/therapeutic use ; Humans ; Leukotriene Antagonists/therapeutic use ; Mast Cells/metabolism ; Mastocytosis/*complications/drug therapy

In up to 40% of systemic mastocytosis (SM) cases, an associated clonal hematological non-mast cell lineage disease such as AML is diagnosed before, simultaneously with, or after the diagnosis of SM. A 40-yr-old man was diagnosed with AML with t(8;21)(q22;q22). Mast cells were not noted at diagnosis, but appeared as immature forms at relapse. After allogeneic hematopoietic stem cell transplantation (HSCT), leukemic myeloblasts were not observed; however, neoplastic metachromatic blasts strikingly proliferated during the state of bone marrow aplasia, and finally, aleukemic mast cell leukemia developed. As the disease progressed, we observed serial morphologic changes from immature mast cells with myeloblasts to only metachromatic blasts and atypical mast cells as mast cell leukemia; FISH analysis showed that the neoplastic mast cells originated from the same clone as the leukemic myeloblasts of AML.
Adult ; Bone Marrow Cells/pathology ; Chromosomes, Human, Pair 21 ; Chromosomes, Human, Pair 8 ; *Hematopoietic Stem Cell Transplantation ; Humans ; In Situ Hybridization, Fluorescence ; Leukemia, Mast-Cell/diagnosis/etiology ; Leukemia, Myeloid, Acute/complications/*diagnosis/therapy ; Leukocytes, Mononuclear/pathology ; Male ; Mastocytosis, Systemic/*diagnosis/etiology ; Recurrence ; Translocation, Genetic ; Transplantation, Homologous

Antineoplastic Agents ; therapeutic use ; Diagnosis, Differential ; Female ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Interferons ; therapeutic use ; Leukemia, Mast-Cell ; metabolism ; pathology ; Mastocytosis, Cutaneous ; metabolism ; pathology ; Mastocytosis, Systemic ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; Middle Aged ; Proto-Oncogene Proteins c-kit ; metabolism ; Radiography ; Radionuclide Imaging ; Spleen ; pathology ; surgery ; Splenectomy ; Tryptases ; metabolism

Female ; Humans ; Infant ; Mastocytosis, Systemic ; diagnosis ; pathology ; therapy

A 4 years-old girl was diagnosed with aggressive systemic mastocytosis at 2 months after the end of chemotherapy including cisplatin, bleomycin and etoposide for an ovarian germ cell tumor (GCT). She was shown pigmented skin lesion, hepatosplenomegaly, thrombocytopenia and increased mast cells with positive toluidine blue staining on the bone marrow examination. Immunohistochemical staining for c-kit was highly expressed in the bone marrow and skin, but not in the GCT specimen. However, c-kit point mutation was detected in the bone marrow, peripheral blood and GCT tissue when performing PCR via oligonucleotide sequencing. We report here on one case of aggressive systemic mastocytosis following ovarian germ cell tumor with c-kit point mutation.
Bleomycin ; Bone Marrow ; Bone Marrow Examination ; Child, Preschool ; Cisplatin ; Drug Therapy ; Etoposide ; Female ; Germ Cells* ; Humans ; Mast Cells ; Mastocytosis, Systemic* ; Neoplasms, Germ Cell and Embryonal* ; Point Mutation ; Polymerase Chain Reaction ; Skin ; Thrombocytopenia ; Tolonium Chloride

The effects of anti-allergic drugs on intestinal mastocytosis and the expulsion of Neodiplostomum seoulense were observed in Sprague-Dawley rats, after oral infection with 500 metacercariae. The drugs used were hydroxyzine (a histamine receptor H1 blocker), cimetidine (a H2 blocker), cyclosporin-A (a helper T-cell suppressant), and prednisolone (a T- and B-cell suppressant). Infected, but untreated controls, and uninfected controls, were prepared. Worm recovery rate and intestinal mastocytosis were measured on weeks 1, 2, 3, 5, and 7 post-infection. Compared with the infected controls, worm expulsion was significantly (P < 0.05) delayed in hydroxyzine- and cimetidine-treated rats, despite mastocytosis being equally marked in the duodenum of all three groups. In the cyclosporin-A- and prednisolone-treated groups, mastocytosis was suppressed, but worm expulsion was only slightly delayed, without statistical significance. Our results suggest that binding of histamine to its receptors on intestinal smooth muscles is more important in terms of the expulsion of N. seoulense from rats than the levels of histamine alone, or mastocytosis.
Animals ; Cimetidine/pharmacology ; Cyclosporine/pharmacology ; Histamine H1 Antagonists/*pharmacology ; Histamine H2 Antagonists/*pharmacology ; Hydroxyzine/pharmacology/therapeutic use ; Immunosuppressive Agents/*pharmacology ; Intestinal Diseases, Parasitic/*drug therapy ; Mastocytosis/*drug therapy/parasitology ; Prednisolone/*pharmacology ; Rats ; Rats, Sprague-Dawley ; Support, Non-U.S. Gov't ; Trematoda/*growth & development/metabolism ; Trematode Infections/*drug therapy

We herein report a case of mastocytosis with unusual cutaneous manifestations resembling pseudoxanthoma elasticum or xanthoma which were exaggerated on the intertriginous areas including axillae, groin and neck in a 39-year-old woman. It was characterized by multiple yellowish papules and plaques with a cobblestone appearance. In addition, there were diffuse red-dish macules and papules of varying sizes mainly on the trunk and extremities. Histopathologic findings showed a very dense infiltrate of large numbers of round to oval cells in the upper and mid dermis. These cells revealed metachromasia on the toluidine blue stain. She also had hepatosplenomegaly and some evidence of bone marrow involvement. There was significant improvement in,the skin lesions with psoralen plus ultraviolet A(PUVA) therapy and potent topical corticosteroid.
Adult ; Axilla ; Bone Marrow ; Dermis ; Extremities ; Female ; Ficusin ; Groin ; Humans ; Mastocytosis ; Neck ; Pseudoxanthoma Elasticum ; PUVA Therapy* ; Skin ; Tolonium Chloride ; Xanthomatosis