the spleen Increase which the part of patients can ustanovit is independent, is important early and conducting a symptom whole rjada gematologicheskih and negematologicheskih diseases and necessarily demand tshchatelnogo reason finding-out. Tselju this message is to present the most important reasons splenomegalii s the clinical point of view and to instruct on carrying out differential diagnoza. While today more and more depart from blind or laparoskopicheskih methods of diagnostics of the reason splenomegalii, invariably big diagnosticheskoe research of dab of blood and a puncture kostnogo a brain continue to save value. They should be used more also by doctors negematologicheskoj specialisations as defined konstelljatsii pictures krovi and a bone brain so are characteristic, that they even as «the diagnosis at a sight» pozvoljajut quickly to define the reason of increase in a spleen. Anatomic and functional preliminary remarks Normalnaja the spleen of the adult weighs about 150-170 Intraperitonealno laying organ in norm completely is latent by the left bottom edges. The spleen is palpated lish then when increases in 1 1/2 - 3 times of more norm (that is weighs okolo 400. For revealing not-big spleen increases, clinical znachenie which it is disputable, special hardware methods of research, takie, for example, as ultrasonic diagnostics are required. V quality of the top border of norm for sonograficheski the defined size selezenki in the majority of textbooks sizes 11 h 5 sm (length H a thickness) are given. Izmerennye sizes are necessary to result in the ratio with the years and body weight, velichinoj bodies of the patient. As expression connected with the years increased immunologicheskoj reactance, at 5-15 % of children and at 3 % of adults without everyone zabolevanija can meet spleen increase. At strong ishudanii or leptosomnom Habitus even at adults it can sometimes be palpated normal selezenka. the Spleen is switched on in blood circulation system limforetikuljarnyj body which at least carries out 4 problems :

and the general instructions on carrying out differentsialnogo the diagnosis splenomegalii Pri palpatsii it is necessary to pay attention of Research of a spleen that the spleen at deep inspiratsii acts slantwise forward-downwards while excursions at breath pecheni more occur in a vertical direction. Respiratory displacement selezenki is the major differentsialno-diagnostic criterion in otnoshenii differentiations from other sort of volume processes in top left podreberi (for example, tumours of kidneys, tumours or kisty a pancreas). Kak the reliable technics of revealing of small increases in a spleen has recommended sebja research of the patient laying on right to the side. Pri infectious mononukleoze and a malaria palpatsiju to spend follows very much ostorozhno as already at the minimum trauma spleen rupture is possible. At nejasnom sensation or at adiposity, as a rule, it is necessary to rely on ultrazvukovoe research which not only will define the exact sizes, but takzhe will give representation about structure of communications of a spleen. For revealing additional dolek spleens (an additional spleen) which is available for 10-35 % of people both in vide a separate segment and in the form of plural in area of gate of a spleen, nozhki spleens or in retropetonealnoj a connecting fabric, sonografija however ne approaches. Reliable possibility of revealing give stsintsiografija spleens or kompjuternaja a tomography. Opredelnnye the conclusions about the reason splenomegalii can be made on the basis dannyh clinical research. It is necessary to pay special attention on konsistentsiju and body size. Obychno it is connected by that infectious or inflammatory hypostases of a spleen chashche all happen a soft consistence while those called neoplasticheskimi processes or ekstramedulljarnym gemopoezom happen dense. Plotnye, hard spleens specify more often on is long current diseases. Takzhe and the spleen size gives the important differentsialno-diagnostic instructions. Chrezmernye spleen increases (huge spleens) are almost always caused hronicheskimi mieloproliferativnymi by diseases, takmi for example, as hronicheskaja mieloidnaja a leukaemia or osteomieloskleroz. After ascertaining uvelichennoj spleens the doctor should carry out purposeful research of others vedushchih symptoms (for example increase in lymph nodes, gepatomegalija, / rpechenochno-skin signs) or clinical symptoms which have big znachenie for diagnostic distribution splenomegalii.

Table 1. the Differential diagnosis splenomegalii

the Differential diagnosis splenomegalii covers both gematologicheskie and negematologicheskie diseases (tab. 1). Almost always spleen increase javljaetsja expression above had disease process. Isolated dobrokachestvennye or malignant new growths of a spleen for example gamartromy, gemangiomy or gemangiosarkomy are extremely rare and almost do not play nikakoj a role in daily clinical practice. Surprises also small chastota metastazirovanija in a spleen (2-3 %) though the blood-groove most part selezenki passes through Pulpastrange and there can sometimes be late on neskolko hours. Stagnant splenomegalija Alkogolnotoksichesky and postgepatitichesky liver cirrhoses it is the most frequent prichina splenomegalii. The spleen hypostasis thus is increase expression davlenija in vorotnoj arteries (a portal hypertensia) and occurrence skin kollateralej i varitse a gullet is frequent assotsiiruet with drugimi changes, such as meteorizm, astsit. There is only moderate increase in a spleen more often. As sledstvie gipersplenizma even at not bleeding patients the anaemia and a thrombocytopenia while values of leukocytes remain normalnymi can develop umerennaja. Along with a cirrhosis and other diseases leading to increase portalnogo of arterial pressure, can proceed with spleen increase. Primerami it the thrombosis of a portal artery, syndrome Budd-Chiari (tromboticheskoe overlapping of veins of a liver) or others ekstragepaticheskie zabolevanija, the blood circulations caused by infringement are. In the end it is possible to name tromboz veins of a spleen which, for example, can develop after a pancreatitis or kak tromboembolicheskoe complication at patients with solid tumours or mieloproliferativnym a syndrome. However a spleen vein thrombosis not necessarily privodit to splenomegalii. In one of researches spent in Chfrite Berlin tolko at 42 (48 %) from 88 patients with chronic pankretaitom and perkrytiem veins selezenki took place spleen increase. Zastojnaja splenomegalija can be called also venous stagnation before right serdtsem, for example, at pravoserdechnoj insufficiency or at Perikarditis constrictiva (kardialnaja a stagnant spleen). Infectious diseases Vospalitelno the caused increases in a spleen can meet at whole rjade infectious diseases (tab. 2).

Table 2. the Reasons infectious caused splenomegaly

At a bacterial sepsis sonograficheski almost vsegda comes to light from easy to moderated splenomegalija which, however, because of svoej a soft consistence is rare can is probed. Patologoanatomicheski poverhnost spleens has a grey-red surface of a cut, with which in bolshom quantity snmaetsja «Pulpabrei» pulpoznaja kashitsa. A triad: subfebrilnaja temperatura, noise in heart and splenomegalija - should always force to think about endokardite . Others important differential diagnozami febrilnogo spleen increases are become rare at us (means in Germany) miliarnyj a tuberculosis and Typhus abdominalis . Naibolee the frequent reason splenomegalii at young men is called by a virus Ebstein-Barr infectious mononukleoz which it is easy diagnostsiruetsja in the presence of characteristic kliniche-skoj pictures (Angina tonsillaris, anikterichnyj a hepatitis), revealing atypical mononuklearnyh cages («Virizyten») in differentsialnom dab of blood and carrying out serologicheskih tests (an IgM-antibody protiv a virus kapsid-antigene of virus Ebstein-Barr, geterofilnyh IgM-antibodies protiv Hammelerythrozyten = reaction Paul-Bunnel). Vo times of distant tourism at us have got value as the reason splenomegalii as well paraziternye the reasons. Prototypes are a malaria and Kala-Azar, transferred on the person mosquitoes (Anophelesmucke) or flies semejstva Phlebotomus. Preobladajushchej the form a malaria in Germany it Malaria tropica, which because of the uncharacteristic current of a fever and changeable klinicheskoj pictures sometimes diagnostsiruetsja only in a stage multiorgannoj nedostatochnosti (a coma, sharp nephritic insufficiency, respiratory nedostatochnost, tsirkuljatornaja insufficiency). The basic find at Malaria tropica is the thrombocytopenia. At a sharp malarial infection not immunizirovannyh travelling on tropics the spleen increase not tak is expressed more often as at chronically-retsidivirujushchih diseases of local population in maljarija-endemichnyh areas. Vistseralnyj lejshmanioz , having long inkubatsionnyj the period (3-6 months), is characterised expressed gepatosplenomegaliej, generalizirovannym by increase in lymph nodes, lihoradkoj, pantsitopeniej and gipergammaglobulinemiej. Podtverzhdenie diagnoses of both diseases it is carried out gematologicheskimi metodami: at a malaria at revealing intraeritrotsitarnogo plazmodija in dab perifericheskoj blood, at Kala-Azar at allocation amastigotnyh lejshmany in retikuljarnyh cages of a bone brain. Granulematoznye inflammations and autoimmunnye Pri carrying out of the differential diagnosis should be considered diseases also not infectious zabo-levanija such as sarkoidoz and kollagenozy. The spleen increase is marked u almost 10-20 % of patients with Boeck. Diagnoz sarkoidoza is based on typical radiologicheskih displays, histologic revealing nekazeoznyh / repitelioidno-cellular granulem in lungs and a liver, substantial growth T-helper limfotsitov in bronhialveoljarnom lavazhe (CD4/CD8 koefitsient > 5), and also povyshennoj activity angiotensin-1-converting the en-winter (ASYA) in syvorotke. S almost same frequency comes to light spleen increase at sistemnoj red volchanke . About system red volchanke neobhodimo to think first of all at young women when simultaneously are available neobjasnimye attacks of a fever with politopnym organnym defeat. The diagnosis podtverzhdaetsja by obnaruzhdeniem antibodies against two-spiral DNA in whey i presence of other ARA-criteria (criteria American Rheumatism Assiciation). Takzhe at syndrome Sharp («mixed connective tissue disease») which it is characterised immunologicheski by revealing of antibodies against ekstrargiruemogo nuklearnogo antibodies, it can be observed splenomegalija. In that vremja as revmatoidnyj the arthritis at adults very seldom proceeds with increase selezenki, Still the syndrome children`s age gives znachitelnoe prevalence vistseralnyh symptoms in shape gepatosplenomegalii and uvelichenija lymph nodes. Osoboj the form of adult age shown by spleen increase, limfadenopatiej, and also granulotsitopenijaej is a Felty-syndrome , vstrechajushchijsja at almost 1 % of all patients with a rheumatic arthritis. Immunologicheskimi leading displays are revmafaktory a high caption, opredelenie antinuklearnyh the antibodies reacting with membrannymi by structures granulotsitov, and also frequent association with HLA-DR4. Lejkopenija, genez which else tochno it is not known, can lead to frequent bacterial infections, that javljaetsja the indication for splenektomii. For 50 % of patients with syndrome Felty in kostnom a brain is available heavy naurshenie maturing granulotsitov, that should vyzyvatsja immunologicheski. Illness of accumulation V quality of other rare reason splenomegalii should name group bolehnej nakoplenija. The most important picture of disease from the therapeutic point of view javljaetsja illness Gaucher , autosomalno-retsessivno nasleduemoe infringement lipidov, leading to accumulation pathological tserebrozidov in macrophages retikuloendotelialnoj systems. Leading symptom javljaetsja slowly progressing, painless tumour of a spleen which mozhet to reach the extreme sizes. Drugie disease signs switch on yellowish or ohrjanaja colouring skin porokovo, pains in bones and deformation of bones. Diagnoz proves to be true biopsijnym research of a bone brain and a spleen, in kotoryh reveal patognomonichnye cages Gaucher, whose pale blue cytoplasm imeet it is g entle-fibriljarnuju structures At. At replacement absent enzima in nastojashchee time probably causal therapy. Amiloidozy , characterised ekstratselljuljarnym otlozheniem fibrilljarnyh proteins (b-Fibrillosen ») and which on structure otkladyvaemogo a fibrous material are subdivided into 6 subgroups, can also vyzyvat splenomegaliju. The spleen is usually moderately increased. The syndrome gipersplenii and spleen rupture is represented by rare complications. At mieloma-assotsiirovannom Al-amiloidoze the clinical picture comes to the end polinejropatiej, a syndrome karpalnogo the tunnel, makroglissiej, to changes of a skin and poliartropatiej. Aa-amloidozy (not an antibody-amiloid) developing in the form of accompanying zabolevany at chronic inflammatory processes, are usually shown nefroticheskim by a syndrome. The diagnosis proves to be true by revealing of adjournment amiloida in a fabric, usually at a capture biopsii a rectum. Haemolitichesky anemias Nezavisimo from calling patomehanizma at each form gemoliticheskoj anemias mozhet to develop splenomegalija. It is based on the worker giperplazii red pulpy. Young age of patients, retsidivirujushchie jaundice attacks, polozhitelnyj the family anamnesis always call suspicion on naichie korpuskuljarnoj gemoliticheskoj anemias. At, in our widths most rasprostranennoj the form, hereditary sferotsitoze the spleen increase okazyvaetsja almost obligatnym a status also meets almost at 90 % of patients. Velichina to a spleen tumour it is almost parallel to size gemoliza. Diagnoz proves to be true typical morphology eritrotsitov in dab peripheral krovi and definition of the lowered osmotic resistance sometimes raised, and korigiruemym glucose autogemolizom. The spherical form pathological eritrotsitov calls that. That in other the usual central enlightenment blood telets more is not defined. Esli it is found out in more adult till now healthy patients voznikaet necessity of carrying out of test Coombs, to exclude autoimmuno-gemoliticheskuju an anaemia at which also in blood dab are defined sharoobraznye cages. Autoimmunogemolitichesky anemias are caused nepolnymi by thermal antibodies of type IgG more often and can meet without distinguished prichiny (idiopaticheski) or again at various diseases (malignant limfomy, tiomy, kollagenozy, Colitis ulcerosa, infections called by a virus Ebstein-Barr and mycoplasmas) after an exposition to medical products. V the plan of the basic steps of diagnostics and differential diagnoza gemoliticheskih diseases is presented table 3.

Table 3. Dostovernost various methods of research for the differential diagnosis gemoliticheskoj anemias

Sharp leukaemias V difference from opinion of textbooks, at patients with sharp leukaemias rather redko is marked palpated increase in a spleen. At the most frequent form lejkemii adult age, sharp mieloidnoj (AML) frequency splenomegalii makes leukaemias about 25 %. On own experience (231 patient with sharp mieloidnoj the leukaemia) splenomegalija marked even only in 15 %. At patients with ostroj lymphatic (ALL) or a sharp not differentiated leukaemia (AUL) frequency splenomegalii a little above also makes, according to German research ALL, tochno 40 %. Diagnoz a sharp leukaemia should lean as well against others clinical projavlenija. Terapevticheski significant differentiation sharp mieloidnoj leukaemias and ostroj a lymphatic leukaemia occurs on the basis of cytologic, tsitohimicheskih and immunologicheskih harak-teristik cages of a bone brain. Bolshoe value therefore is given to spleen increase at diagnosis statement i the differential diagnosis chronic mieloproliferativnogo a syndrome. Hronicheskaja mieloidnaja a leukaemia (CML) and osteomieloskleroz are obligatnymi pri increase in a spleen which on occasion can reach huge razmerov. Such huge spleens at autopticheskih researches can dostigat weight of 10 kg. Takzhe and at Polycythaemia vera almost at 80 % of patients already at diagnosis statement vyjavljaetsja splenomegalija. Therefore it vera has been entered by the American group of studying Polycythemia as the basic criterion into the differential diagnosis independent and vtorichnogo eritropoezov. Unlike chronic mieloidnoj leukaemias and osteomieloskleroza there is only moderate increase in a spleen more often. Znachitelnoe the increase in the size of a spleen during disease speaks for perehod Polycythaemia vera in mielofibroticheskuju a stage. Essentsialnaja trombotsitemija differs from other forms mieloproliferativnogo a syndrome that v the beginning very seldom is available splenomegalija. Razumeetsja, that the spleen status represents only differentsialno diagnostichesky criterion. Exact nozologicheskoe definition of a picture of disease predpolagaet carrying out careful gematologicheskoj diagnostics including a picture krovi and differential dab, definition alkaline fosfatazy leukocytes, tsitologicheskoe and histologic research of a bone brain, and also tsitogeneticheskuju and sometimes molekuljarno-genetic diagnostics. Mielodisplastichesky the syndrome (MDS) seldom proceeds with spleen increase. On dannym the big modular statistical researches it meets only at 12-21 % of patients with mielodisplas ticheskim a syndrome. The exception is represented hronicheskaja mielomonotsitarnaja by a leukaemia (CMML) characterised by increase monotsitarnyh prestages in blood and a bone brain and on clinical and gematologicheskim to criteria occupies intermediate position between mielodisplasticheskim and chronically-mieloproliferativnym syndromes. U 33-70 % of patients with chronic mielomonotsitarnoj a leukaemia at statement diagnoza are marked splenomegalija. «the huge spleen» with a syndrome gipersplenii or semiology abdominalnyh bolej (retsidivirujushchie heart attacks of a spleen, display gastrointestnalnyh zameshcheny) In rare instances can even develop. From chronic mieloidnoj leukaemias the leukaemia among other absence of the Fila-Delfijsky chromosome ili a BCR-ABL-hybrid gene differs chronic mielomonotsitarnaja. Malignant limfoma Takzhe and for heterogeneous group of diseases malignant limfomy revealing splenomegalii has important diagnostic, differentsialnodiagnosticheskoe and prognosticheskoe value. Though concerning Hodgkin and Non-Hodgkin limfom uvelichenie spleens is the important clinical parametre for a stage estimation rasprostranenija diseases or weights of a tumour. S expansion applied classifications limfom had appeared today, that especially nekotorye variants is low-malignant Non-Hodgkin-limfomy can proceed with osobenno expressed splenomegaliej. To takmi the leukaemia (PLL) concerns immunotsitoma which at nebolshogo percent of patients is limited to a spleen, volosinchato-cellular lejkemija (HZL) and prolimfotsitarnaja. V the big Italian research for more than 85 % of patients with / a rvolosinchato-cellular leukaemia at potsanovke the diagnosis already was available splenomegalija, at 25 % of patients the spleen left for edge of a costal arch on more chem 10 see In the relation prolimfotsitarnoj leukaemias it is marked typically nesootvetstvie between massive splenomegaliej and absence or malovyrazhennostju increase in lymph nodes. Diagnoz a volosinchato-cellular leukaemia and prolimfotsitarnoj leukaemias it is frequent stavitsja on the basis of dab of peripheral blood. As for patients with / a rvolosinchato-cellular leukaemia often is available lejkopenija, it is necessary naznachit Buffy Coat - a preparation to facilitate revealing volosinchatyh cages. At volosinchatyh cages there are fringed vyrosty cyto-plasmas, and tsitohimicheki they harakterizujutsja tartrat-resistant sour fosfatazoj. And on-turn, at patients s prolimfotsitarnoj the leukaemia, as a rule, marks the raised quantity lejkotsitov (to 400 000/ml). Tumoral cages more than at chronic limfaticheskoj also pay attention of a leukaemia expressed ("vodnoprozrachnoj") solitarnoj Nukleolus. Immunotsitologicheski it is possible to differentiate B-PLL (high density membrannyh immunogemoglobinov) and T - cellular variants (primary expression CD4). Other extremely rare edinitsoj often proceeding with splenomegaliej is lienalnaja limfoma with villoznymi limfotsitami (splenic lymphoma with villous lymphocytes, SLVL). This disease which should concerns to limfotsitarnym limfomam along with the expressed tumour of a spleen is characterised moderately povyshennymi by quantities of leukocytes (to 40 000/ml), circulating V-limfotsitami s morphology similar volosinchatym to cages (from-nositelnoe quantity of 40-100 %) a also is frequent monoklonalnoj gammopatiej (60 % of patients). Also in morfologicheskom a phenotype there are crossings with volosinchatokletochnoj a leukaemia, odnako CD11c - and CD25 - the antigene at SLVL is negative. the Source: C. Aul, U. Germing, G. Meckenstock und A. Heyll. Differentialdiagnostische Uberlegungen zum Problem der Splenomegalie. Padiat.prax. 54, 69-82. Perevod from German - J.M.Bogdanov, chair of pediatrics FPK Northern meduniversiteta

Article is published on a site http://medolina.ru /