HELLP syndrome

Template:Infobox - onemocněníHELLP syndrome is a very serious complication of pregnancy with significant mortality and morbidity (up to 40%). The name originated from the English words Hemolysis, Elevated Liver enzymes, Low Platelets . It is described either as a separate disease or as a complication of preeclampsia . It is a state of severe preeclampsia complicated by hemolysis , thrombocytopenia , abnormal peripheral blood smear, and abnormal liver function tests .

Incidence

In 2-12% of preeclampsia, most often in multiparous couples around the age of 25, most often in the 36th week, in 70% before delivery, in 30% after delivery.

Pathogenesis

Unclear, probably generalized vasospasm with subsequent MODS (mainly liver , kidney ) and activation of coagulation . The result is microangiopathic thrombocytopenia .

Laboratory

thumb|right|Schistocyt

- Hemolysis – pathological smear of blood – there are schistocytes and burr cells (triangular erythrocytes ) – probably formed by flow through the affected vessel, rise of bilirubin above 20 μmol/l, LD (5×), free iron in the serum.
- Elevation of liver tests – mainly AST (2–3×).
- Thrombocytopenia - drop below 100, consumption and destruction.

Klinický obraz

Dominuje bolest v epigastriu a v pravém podžebří, nauzea a zvracení. Prodromy – nespecifické, chřipkovité (malátnost, únava). Pokročilé stádium se projevuje krvácením (hematurie, krvácení do GIT). Hmotnostní přírůstek s otoky, hypertenze s proteinurií, někdy ikterus. Plně rozvinutý HELLP imituje DIC.

Diferenciální diagnostika

Akutní steatóza jater, ITP, HUS, hyperemesis gravidarum, cholecystopatie, virová hepatitida, gastroenteritida, apendicitida, pyelonefritida, renální kolika, SLE.

Komplikace

DIC (až u 40 %), abrupce placenty, akutní respirační insuficience, ARDS, ascites, ruptura subkapsulárního hematomu jater, eklampsie, IUGR.

Léčba

Ukončení gravidity je jedinou kauzální léčbou.^[1] Konzervativní terapie je pro poporodní období. Expanze plasmatického volumu, úprava koagulace (antitrombin III, plasma, erymasa, trombocyty), kortikoidy (imunosuprese), protikřečová profylaxe (MgSO₄, valium), antihypertenziva, ATB, dieta, hepatoprotektiva, plasmaferéza (dialýza).

Odkazy

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