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espanolEsta actualizacion aborda el grupo de trastornos que van a afectar a las paredes de los vasos sanguineos y a las plaquetas, que son parte fundamental de la hemostasia primaria. Se dividen en purpuras (afectacion vascular) o alteraciones cuantitativas o cualitativas de las plaquetas. Pueden ser congenitas o adquiridas. La incidencia de muchas de estas entidades va a ser baja, considerandose algunas como enfermedades raras. Las manifestaciones hemorragicas tipicas van a ser la aparicion de sangrados cutaneomucosos con los traumatismos. Algunas de las coagulopatias congenitas van a formar parte de grupos sindromicos en los que la afectacion va a ir mas alla de la clinica hemorragica, requiriendo un abordaje multidisciplinar. En toda coagulopatia es importante realizar una historia clinica personal y familiar minuciosa. En este apartado, son utiles las pruebas de funcionalidad y agregacion plaquetaria. Cada vez mas se estan conociendo las alteraciones geneticas que hay detras de estos cuadros, cobrando mas importancia las tecnicas de diagnostico molecular. En las formas congenitas, el tratamiento va a ser sintomatico, dirigido segun la localizacion y acompanado de equipos multidisciplinares. Para los casos con afectacion de la funcion de las plaquetas, ademas se podra optar por la transfusion de plaquetas. EnglishDiseases of primary hemostasis. Vascular purpura. Platelet disorders: This update addresses the group of disorders that affects blood vessel walls and platelets, which are a fundamental part of primary hemostasis. They are divided into purpura (vascular involvement) or quantitative or qualitative platelet abnormalities and can be congenital or acquired. The incidence of many of these entities is low; some are considered rare diseases. The typical hemorrhagic manifestations include the appearance of cutaneous and mucosal bleeding with trauma. Some congenital coagulopathies form part of groups of syndromes in which involvement goes beyond bleeding symptoms, requiring a multidisciplinary approach. In all coagulopathies, it is important to take a thorough personal and family medical history. In this regard, platelet function and aggregation tests are useful. More and more, the genetic alterations behind these cases are becoming known, with molecular diagnostic techniques gaining greater importance. In congenital forms, treatment is symptomatic, targeted according to the localization, and accompanied by multidisciplinary teams. For cases with platelet function involvement, a platelet transfusion can also be opted for.

Introduction and importance The SARS COV2 infection is a challenging pandemic that has affected millions of people with a very high mortality rate. In addition to the typical respiratory symptoms, it can also cause variable skin lesions, such as vascular purpura in some exceptional cases. Case presentation We report the case of a 60-year-old woman who was admitted for a SARS COV2 infection, the evolution was marked by the appearance of a vascular purpura at D20 after the beginning of the symptoms. Discussion The cutaneous manifestations associated with the SARS COV2 infection are polymorphic. Vascular purpura is one of them. Its diagnosis is retained in the light of a combination of arguments, which makes it a real challenge for the physician to diagnose it. The management of the disease is based on a symptomatic treatment. The clinical evolution is, in general, favorable. Conclusion Although rare and still not fully explained, skin involvement during SARS COV2 infection has been described. It should not be neglected and it should be diagnosed early and treated appropriately, especially in asymptomatic patients.

Le purpura hypergammaglobulinémique de Waldenstrom (PHGGW) est un syndrome caractérisé par un purpura vasculaire récidivant associé à une hypergammaglobulinémie. Il peut être isolé ou plus fréquemment associé à une hémopathie maligne ou à une connectivite essentiellement le syndrome de Gougerot Sjögren (SGS). Patiente de 48 ans suivie depuis 2 ans pour un SGS associé à un lupus érythémateux systémique, qui présentait depuis environ 6 mois une éruption cutanée paroxystique et récidivante des membres inférieurs s'aggravant à l'effort. Il ne s'y associait pas de fièvre, de sueurs ou d'altération de l'état général. A l'examen cutané, il existait aux membres inférieurs des lésions érythémateuses, pétéchiales, infiltrées et ne s'effaçant pas à la vitropression évocatrices de purpura vasculaire. Le reste de l'examen était sans anomalies. A la biologie, il existait une lymphopénie à 1280/mm3, une élévation de la vitesse de sédimentation à 112 mm H1 et une hyperprotidémie à 108g/l en rapport avec une hypergammaglobulinémie d'allure polyclonale à 68g/l. Les valeurs de la CRP et de la fibrinémie étaient normales. Le dosage du complément sérique était normal. La cryoglobulinémie était négative ainsi que les sérologies de l'hépatite B, C, CMV et HIV. L'échographie trans-thoracique n'objectivait pas de signes d'endocardite infectieuse. Le diagnostic de PHGGW a été retenu devant l'association d'un purpura récidivant à une hypergammaglobulinémie et en l'absence d'éléments en faveur d'une autre étiologie à savoir une cause infectieuse, une cryoglobulinémie ou une vascularite secondaire. La patiente a été traitée par de la colchicine à 1mg/j avec une nette amélioration.Pan African Medical Journal 2015; 22

Resume Les methodes de dosage et d'analyse des cryoglobulines ont beaucoup progresse ces dernieres annees notamment par une meilleure maitrise de la phase preanalytique et la standardisation des nombreuses etapes de l'analyse. L'information apportee par cet examen est importante en medecine interne dans l'aide au diagnostic etiologique de certaines manifestations cliniques (purpura vasculaire, syndrome de Raynaud) ou pour orienter vers un diagnostic impliquant la production des immunoglobulines (hemopathies lymphoides, maladies virales ou auto-immunes). Nous decrivons un protocole technique, detaille, tres aisement transposable dans de nombreux laboratoires. Ce protocole assure une realisation optimale de cet examen en clarifiant tous les facteurs generateurs de resultats aleatoires. Les precautions preanalytiques, les choix techniques et les principes de l'interpretation preconises peuvent etre rapidement mis en œuvre sans investissement disproportionne. Les recommandations proposees sont coherentes avec la demarche qualite appliquee au laboratoire.

Resume Le scorbut atteint preferentiellement mais pas uniquement les sujets âges et les exclus, par consommation insuffisante de fruits et legumes frais. Les manifestations cliniques sont une asthenie, un purpura vasculaire, un syndrome hemorragique, une atteinte stomatologique. Les manifestations articulaires sont presentes dans 80 % des cas : arthralgies, myalgies, hemarthroses associees a des hematomes intramusculaires. La depletion vitaminique entraine une alteration de la structure du collagene, un defaut de formation de la matrice osteoide et une augmentation de la resorption osseuse. Les signes radiologiques sont varies : osteolyse avec parfois pincement articulaire, osteonecrose, osteopenie, proliferation periostee. L'osteoporose trabeculaire et corticale est frequente. Chez l'enfant, le scorbut est responsable de douleurs intenses des membres inferieurs liees a des hemorragies sous-periostees. Les signes biologiques sont non specifiques : anemie, hypocholesterolemie, hypoalbuminemie. Le diagnostic est confirme par le dosage d'ascorbemie (inferieur a 2,5 mg/l). L'administration de vitamine C corrige rapidement les symptomes.

We present a case of vascular purpura revealing an intra-cardiac left-sided thrombus complicating an end-stage dilated cardiomyopathy. Vascular purpura main etiologies encompass the wide spectrum of vasculitides and microvascularocclusion syndromes. Among them, cardiac embolism represents an unusual but potentially severe etiology.

Resume Introduction Le lymphome splenique a lymphocytes villeux est parfois satellite d’une infection chronique par le virus de l’hepatite C. Il a ete recemment rapporte que le traitement contre l’hepatite C pouvait etre aussi efficace vis-a-vis du lymphome. Nous en rapportons un nouveau cas, revele par des lesions cutanees. Observation Un homme de 53 ans consultait pour un purpura vasculaire des jambes dans un contexte d’arthralgies et de neuropathie des membres inferieurs. L’hemogramme objectivait une hyperlymphocytose composee de lymphocytes villeux faisant porter le diagnostic de lymphome splenique a lymphocytes villeux. La biopsie cutanee trouvait des thromboses des vaisseaux dermiques superficiels rapportees a une cryoglobulinemie satellite d’une infection chronique a virus C. La cryoglobulinemie etait compliquee d’une atteinte renale et neurologique. Le malade etait traite par interferon, ribavirine et echanges plasmatiques avec un bon resultat initial. Discussion Le purpura vasculaire, souvent associe aux cryoglobulinemies, peut etre revelateur d’une hepatite C. L’efficacite du traitement par interferon et ribavirine, vis-a-vis du lymphome splenique a lymphocytes villeux associe a une hepatite C, a ete recemment documentee. Il permet d’obtenir une remission des manifestations de la cryoglobulinemie chez 78 p. 100 des malades, parallelement a la negativation de la charge virale, et parfois la remission du lymphome.

Sir,The combination of chloroquine, an amino-4-quinoleine,and proguanil, a biguanide derivative, is widely usedfor antimalarial prophylaxis by travellers in countriesof high endemic prevalence of Plasmodium falciparumchloroquino-resistance (1).Among numerous adverse effects of these two drugs,vascular purpura due to chloroquine has been men-tioned only once (2). Since then, no case report ofvasculitis induced by proguanil or chloroquine hasever been published. We report a case of severe cuta-neous necrotizing vasculitis induced by Savarine

We report a patient who developed during HIV primary infection a symptomatic mixed cryoglobulinemia. The patient suffered from arthralgias, vascular purpura of the legs, and proteinuria. Cryoglobulinemia progressively disappeared in several months after HAART.

Resume Une observation de purpura vasculaire apres injection de dalteparine au cours d'un cancer bronchique complique de phlebite est rapportee. Le purpura cedait apres arret de la dalteparine, alors qu'aucune autre cause de purpura n'etait mise en evidence. L'intradermo-reaction a la dalteparine positive evoquait un mecanisme d'hypersensibilite, mecanisme conforte par la notion d'une prescription anterieure a la toxidermie de la dalteparine. Un heparinoide de synthese, le lomoparan, put etre substitue a la dalteparine sans intolerance. Cette observation, tres rarement decrite, souligne la necessite d'une surveillance accrue des heparines de bas poids moleculaire. Ces molecules, de plus en plus prescrites dans la maladie thromboembolique, sont pourant a l'origine d'hemorragies et d'effets secondaires immunoallergiques, surtout des thrombopenies mais aussi des toxidermies, comme celle decrite dans notre observation. Les heparinoides de synthese pourraient constituer une alternative therapeutique.

Summary The authors report the first case of cutaneous vasculitis presenting as an acute purpura related to sparfloxacin intake.

We report the case of a 64 year-old woman who had pulmonary fibrosis and sicca syndrome for 4 years and in whom vascular purpura occurred, leading to the diagnosis of C hepatitis related cryoglobulinemia. The nosologic links between diseases are discussed.

A 64 year-old man was found to have a vascular purpura with histologic features of cutaneous necrotizing vasculitis, fifteen days after influenza vaccination. No other cause (neoplasic, autoimmune, infectious or drug-induced) was apparent, and the reponsability of vaccination is retained while it is a very rare complication.

血管性紫斑病について毛細血管抵抗, 出血時間, 血小板数などの検査成績を対照群と比較した。また, Rumpel-Leede法による毛細血管抵抗について検討した。対照群(非紫斑病患者)では30才以上で30%にRumpel-Leede法陽性を示した。単純性紫斑, 女子深在性紫斑, 機械的紫斑では, 疾患相互に検査成績に差はみられず, また同年令層の対照群と比べても差は認められなかつた。AngiodermatitisではRumpel-Leede法陽性例が多いが, 好発年令が高年令者層にあるためと思われる。また, 血小板数は一部の症例を除き正常範囲にあり, 出血時間の病的な延長をみた例はなく, 本症は血管障害性の変化によるものと考えられる。

THE term vitamin P was proposed by Armentano and his co-workers1 to denote a group of compounds distinct from ascorbic acid that maintained the physical strength of capillaries. This concept grew out of investigations to determine why crude sources of vitamin C controlled hemorrhages in vascular purpura whereas purified ascorbic acid was ineffective. From the ascorbic-acid-free residue of the mother substance, a faintly yellow crystalline material, which was found to exert the so-called vitamin P activity, was extracted. Because of its derivation from citrus fruits, this active powder was called citrin. Various analyses revealed that citrin was a mixture of . . .

An instance of nonthrombocytopenic vascular purpura is reported due to tartrazine sensitivity. There were no abnormal hematologic findings present. Possible pathogenetic mechanisms involved in this condition are discussed.

SummaryCharacteristic electrophoretic patterns have been observed in all 5 cases of hemophilia and in all 8 cases of idiopathic thrombocytopenic purpura. These patterns show the following characteristics: (a) appearance of an unusual peak in the group of the α-globulins (αx), and (b) disappearance of the usual α2- and α3-globulin peaks. The same anomaly has been observed in some cases of secondary thrombocytopenia, “anaphylactoid” type of vascular purpura, and hemolytic anemia with evidence of platelet dysfunction.

The clinical course of a patient is described whose primary manifestations of a circulating cryo IgGK were cutaneous symptoms of pain, vascular purpura and necrotic ulcerations, especially of the lower extremities. A biopsy specimen of skin lesions showed vasculitis and immune reactants in the vessel walls. The aetiology was not recognized. The effect of primary long-term therapy with prednisone was not essential. Combined therapy with cyclophosphamide and plasmapheresis allowed a satisfactory initial remission but had to be maintained repeatedly in order to reduce the periodic recurrence of clinical signs and symptoms. Renal failure terminated the clinical course at 25 years from onset of symptoms.

Summary A case of congenital anhidrotic ectodermal dysplasia is presented. The case is of special interest in that the child also had a nonthrombocytopenic vascular purpura.

Excerpt The purpose of this report is to present a case of purpura occurring in an individual receiving therapeutic doses of colchicine for acute gouty arthritis. Search of the literature from the ...

The Schoenlein-Henoch syndrome is described on the basis of 44 personally observed cases. It is defined as a condition in which nontraumatic hemorrhage with or without edema may occur in the skin, or subcutaneous tissue, or joints, or viscera, or in any combination of them, and in which the blood platelets are normally abundant. The causes of the syndrome are varied, but streptococcal infection is important, and "anaphylactoid" causes rare. The prognosis is good, and the 4 cases with renal involvement also did well. Schoenlein-Henoch purpura and purpura simplex overlap, but in contrast with the latter, Schoenlein-Henoch purpura is not familial, and is common in males and children.

PURPURIC reactions resulting from the use of tetracyclines appear to be rare. Oxytetracycline-induced vascular purpura without thrombocytopenia has been reported before, 1 but oxytetracycline-induced thrombocytopenic purpura has not yet been widely reported. Report of a Case A 68-year-old man was admitted to Walton Hospital, complaining of fatigue, loss of appetite, mild abdominal pain, and sickness, which became evident two days before admission, and a purpuric rash covering both legs, which appeared suddenly during the night before admission. He had a mild chest infection the previous week, and the family physician had treated him with oxytetracycline tablets, 500 mg four times daily, six days before admission. No other medication had been prescribed. He had had repeated attacks of bronchitis during the last five years and had taken various antibiotics during these attacks. The last attack was one year previously. His history showed that he had been suffering from ankylosing spondylitis for

To the Editor.— The mechanisms responsible for purpura are diverse and, in some instances, not completely understood. Generally, the causes of purpura can be classified into vascular defects (either primary or secondary), platelet abnormalities, and coagulopathies. 1 One example of secondary vascular purpura is the one that may occur in the loose tissues of the face and neck after violent or prolonged coughing or vomiting or Valsalva's maneuver. 2 Crops of petechiae appear but are of cosmetic importance only. The eruption gradually subsides without treatment. Report of a Case.— A healthy 45-year-old man was examined for an asymptomatic dermatitis of one week's duration that was confined to the periorbital areas, the glabella, and the upper part of the bridge of the nose. The skin lesions were first noted by his physician during a physical examination that included pneometry. Further questioning disclosed that his physician had not noticed the lesions until

Excerpt Since Owren's description in 1948 of aplastic crisis in congenital hemolytic jaundice (1), crises have been demonstrated to occur in hemolytic disorders, including other cases of congenital...