Usefulness of a Simple Immunohistochemical Staining Technique to Differentiate Anti-p200 Pemphigoid From Other Autoimmune Blistering Diseases: A Report of 2 Cases. / Descripción de 2 casos de penfigoide anti-p200. Utilidad de una técnica inmunohistoquímica sencilla en el diagnóstico diferencial con otras enfermedades ampollosas autoinmunes.

Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease characterized by the presence of circulating immunoglobulin G antibodies directed against laminin gamma-1, a 200-kDa protein located in the lamina lucida of the basement membrane. We review the clinical, histopathological and immunological characteristics of the first 2 cases described in Spain. Anti-p200 pemphigoid shares histopathological and immunopathological findings with epidermolysis bullosa acquisita, the main entity in the differential diagnosis. However, its management follows the same guidelines as those used for bullous pemphigoid. The diagnosis is confirmed by immunoblotting, which is a complex technique available in few centers. We propose the immunohistochemical detection of collagen type IV on the floor of the blister, combined with standard immunofluorescence techniques, as a simple, accessible alternative to differentiate anti-p200 pemphigoid from epidermolysis bullosa acquisita.

Cross-Cultural Adaptation of the Urticaria Control Test From German to Castilian Spanish.

INTRODUCTION: The clinical concept of urticaria embraces a heterogeneous group of conditions classified according to their clinical course as acute (lasting less than 6 weeks) or chronic (lasting 6 weeks or more). Chronic urticaria may be either spontaneous or induced. Few tools are available for monitoring the various clinical forms of this disease or for evaluating its impact on quality of life. The recently developed Urticaria Control Test to evaluate disease control is available in German, the original language, and American English. OBJECTIVE: To culturally adapt the long and short versions of the Urticaria Control Test to Castilian Spanish to ensure equivalence between the translated items and those of the original version. MATERIAL AND METHODS: To translate the Urticaria Control Test we followed the International Society for Pharmacoeconomics and Outcomes Research good practice guidelines, starting with forward translation and moving through back translation and cognitive debriefing steps. RESULTS: Three items were modified when the first Spanish version, translated from German, was discussed (cognitive debriefing). The revised translation was then translated back to German and sent to the Urticaria Control Test authors, who modified one item they considered had acquired a different focus through translation. A third Spanish version was then prepared and after minor proofreading changes was considered definitive. CONCLUSIONS: This study was the first step in making it possible to use the Urticaria Control Test questionnaire in Castilian Spanish. The next step will be to validate the translated questionnaire.

[Pure red cell aplasia and neutropenia associated with chronic monoclonal T-lymphocytosis]. / Aplasia pura de serie roja y neutropenia asociadas a linfocitosis T crónica monoclonal.

We report a patient with nonregenerative anemia and neutropenia associated with an increment of circulating large granular lymphocytes (LGL). The anemia was secondary to the absence of erythroid precursors in the bone marrow or pure red blood cell aplasia associated with moderate lymphoid infiltration by LGL. Myeloid (CFU-GM) or erythroid (CFU-E, BFU-E) precursors were not detected by bone marrow culture. A high number of T colonies was found. The lymphoid population had a cytotoxic/suppressor phenotype (CD2+, CD4-, CD8+). Virologic studies (including search for HTLV1) were carried out with negative results. A clonal origin was demonstrated by DNA analysis with probes of those genes encoding T receptor (TRc). After cytostatic therapy with cyclophosphamide and low doses of prednisone a clinical and laboratory remission was achieved. We review the literature, with a discussion of the clinical, phenotypic and molecular features of this disease as well as its response to therapy.

[Beta-thromboglobulin and platelet factor 4 in a follow-up of acute myelofibrosis (megakaryoblastic leukemia)]. / Betatromboglobulina y factor 4 plaquetario en el seguimiento de una mielofibrosis aguda (leucemia megacarioblástica).

We report a patient with acute myelofibrosis (AM) in whom a megakaryocytic origin was demonstrated after conventional microscopy, investigation with monoclonal antibodies directed against the glycoprotein complex IIb/IIIa (CD41a) and the platelet peroxidase (PPO) reaction. Thus, a diagnosis of acute megakaryoblastic leukemia (AMGL) was made. It is now known that this megakaryoblastic proliferation is responsible for myelofibrosis as an increased release of platelet-derived growth factor (PDGF), beta-thromboglobulin (BTG), and platelet factor 4 (PF4) develops because ineffective megakaryocytopoiesis and failure of these clonal populations to store the mentioned substances in their alpha granules. At the time of diagnosis, the plasma concentrations of BTG and PF4 were measured and were found to be high. Thus, an increased PDGF level was indirectly assumed, with the subsequent fibroblast stimulation. After treatment with low dose cytosine arabinoside, a clinical, analytical and histological remission was achieved, with a return of BTG and PF4 values to the normal range. It was therefore concluded that the follow up of these parameters is useful for the diagnosis and the establishment of remission criteria in these patients.

[Beneficial effect of 1,25-dihydroxyvitamin D3 in the treatment of myelofibrosis]. / Efecto beneficioso de la 1,25-dihidroxivitamina D3 en el tratamiento de la mielofibrosis.

Two cases of myelofibrosis are reported, one corresponding to a chronic idiopathic myelofibrosis and another to an acute myelofibrosis or megakaryoblastic leukemia (AMGL). According to current knowledge, the origin of fibrosis in these disorders is a megakaryoblastic/megakaryocytic proliferation in the bone marrow. These megakaryocytic clonal populations result in an ineffective megakaryocytopoiesis and in an inability to store beta-thromboglobulin (BTG), platelet factor 4 (PlF4) and platelet derived growth factor (PDGF) in the alpha granules, whereby their release is increased. Both PDGF, a powerful stimulator of fibroblastic activity, and PlF4, a collagenase inhibitor, cooperate in the development of myelofibrosis. Owing to its action inducing cellular differentiation towards the monocytic-macrophagic line, which secretes collagenases and inhibits megakaryocyte proliferation, treatment with 1,25-dihydroxyvitamin D3 was attempted in both cases, 2.5 micrograms daily for 6 months. Satisfactory results were achieved.

[The reactive hemophagocytic syndrome associated with infection: a study of 3 cases]. / Síndrome hemofagocítico reactivo asociado a infección: estudio de tres casos.

Reactive hemophagocytic syndrome (RHS) or hemophagocytic histiocytosis is a disease with anatomo-pathological features of systemic proliferation of non-neoplastic histiocytes, with prominent hemophagocytosis, associated to infection of other diseases. The cases of three patients afflicted with RHS are presented. 2 of them secondary to a brucellosis and the other of unknown origin. The clinical features were similar: high fever, wasting, and splenomegaly. Pancytopenia existed together with liver disfunction, CID and hyperferremia. Marrow infiltration of reactive histiocytes with important hemophagocytic phenomenon, demonstrated by aspirated and bone marrow biopsies, were observed in all cases. Studies of the immunology system were performed, showing changes in two of them. All of them fully recovered after antibiotic treatment.

[Variant hairy-cell leukemia: immunophenotypic and ultrastructural study of a case]. / Tricoleucemia-variante: estudio inmunofenotípico y ultraestructural de un caso.

The recent introduction of new methods to identify different lymphocytic subsets has made it possible to recognise a rare variant of the classic hairy cell leukaemia, showing intermediate features between prolymphocytic leukaemia and hairy cell leukaemia. A 37-year-old patient is reported who followed a mildly aggressive clinical course and had massive splenomegaly without lymph node enlargement. Moderate leucopenia with lymphocytosis was present, with frequent hairy cells carrying one prominent nucleole. The cytochemical pattern include tartrate-sensitive acid phosphatase positivity, and the immunophenotype of such cells was CD22++, CD11++, CD24-, CD25-, CD2-, CD5-, CD19++. No lamellar ribosomal complex was seen in the ultrastructural study of the hairy cells. The patient was diagnosed as having variant hairy cell leukaemia and achieved partial response after splenectomy. The clinical, diagnostic and therapeutic aspects of this rare variant are discussed.