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Gangguan Perdarahan - Coggle Diagram
Gangguan Perdarahan
Penyakit gangguan perdarahan
Acquired platelet function defects
congenital platelet function defects
disseminated intravascular coagulation (DIC)
prothrombin deficiency
Factor V deficiency
Factor VII deficiency
Factor X deficiency
Factor XI deficiency (hemophilia C)
Glanzmann disease
Hemophilia A
Hemophilia B
Idopathic thrombocytopenic purpura (ITP)
Von Willebrand disease (type I, II, III)
Patofisiologi perdarahan yang sulit berhenti
a) Changes in blood vessels: Vascular disorders are a heterogeneous group of diseases or conditions that are characterized by easy breakage, with consequent bleeding of small vessels (arterioles and capillaries)
B) Platelet Disorders: inherited platelet disorders are a cause of haemorrhagic syndromes, although rare, ranging from minimal to severe bleeding (11). The clinic is similar in all platelet disorders, studying with a purpuric syndrome of cutaneous-mucosal multiple and spontaneous bleeding. We can distinguish two different types of alterations.
Thrombocytopenia
due to changes in production, increased destruction, alterations in the distribution and idiopathic or hereditary illnesses, also called hereditary thrombocytopenia (thrombocytopenia relatives).
there is not a sufficient number of platelets to ensure hemostasis
Thrombocytopathy
primary alterations may be due to defects and / or malfunctions in: the platelet surface glycoproteins, intraplatelets granules; cytoskeletal structural proteins of thrombocytes, catalytic / host / activating activity of the processes of plasmatic phase in the hemostasis or in the transmission systems of messages / signals from the surface to the platelet cytoplasm
Coagulopathies
Plasmatic alterations that cause alterations in the secondary hemostasis process
Congenital disorders
Factor VIII and IX deficiency
impaired coagulation cascade
VWF deficiency
Vit K deficiency
Vitamin K is needed for the processes of synthesis of factors II, VII, IX, and X as well as proteins S and C. Its deficiency causes an increased PT and APTT.
References
Doherty TM, Kelley A. Bleeding Disorders. [Updated 2020 Aug 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from:
https://www.ncbi.nlm.nih.gov/books/NBK541050/
“What is a Bleeding Disorder?”. National Hemophilia Foundation. 2020. Available from:
https://www.hemophilia.org/Bleeding-Disorders/What-is-a-Bleeding-Disorder
Kaushansky, K. et al. Williams Hematology. 9th Ed. New York: McGraw-Hill Education. 2016.
Hall, J. Guyton and Hall Textbook of Medical Physiology. 13th Ed. Elsevier. 2016.
Wahed, A. and Dasgupta, A. Hematology and Coagulation: A comprehensive Review for Board Preparation, Certification and Clinical Practice. Elsevier. 2015.
Doherty TM, Kelley A. Bleeding Disorders. [Updated 2020 Aug 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from:
https://www.ncbi.nlm.nih.gov/books/NBK541050/
Tatalaksana awal pada gangguan perdarahan
severe thrombocytopenia or a platelet functional
defect or both should be treated with platelet transfusions
a single-factor deficiency can be effectively reversed by administering the appropriate purified factor or fresh frozen plasma
Treatment with vitamin K and/or fresh frozen plasma can also be effective in the patient with multiple deficiencies of vitamin K--dependent factors resulting from warfarin or other causes.
As for the DIC patient, stepwise replacement of platelets, fibrinogen, and coagulation factors are temporizing measures to lessen the bleeding;
Terapi Suportif
Hindari luka
Merencanakan tindakan operasi
Mengatasi perdarahan akut dengan REST, ICE, COMPRESSION, ELEVATION
Kortikosteroid dapat menghilangkan proses inflamasi
Analgetika pada pasien hematrosis dgn nyeri hebat
Mekanisme pembekuan darah
Vasoconstriction
Mediated by reflex neurogenic mechanisms
augmented by endothelin, released from damaged endothelial cells
reduces blood flow and extend of blood loss
Platelet plug
platelets adhere to subendothelial collagen, change shape and release contents of its granules
other platelets are recruited, forming a platelet plug
the platelet plug is reversible but it may become irreversible with fibrin
Activation of Coagulation Cascade
activation of the clotting cascade results in the formation of fibrin and cross-linking of fibrin with resultant arrest of bleeding
the coagulation cascade is activated by the appearance of tissue factor (FIII) which is not normally exposed
Tissue factor is presented when a blood vessel is injured
the coagulation cascade consists of intrinsic pathway, extrinsic pathway and common pathway - ending in fibrin and cross-linked fibrin, a stable clot
Diagnosis gangguan perdarahan
Laboratory diagnosis
Pemeriksaan fungsi vaskular
Pemeriksaan rumple leede
Pemeriksaan masa perdarahan dengan metode Duke and Ivy
Pemeriksaan fungsi selular
pemeriksaan jumlah trombosit
Otomatis: electrical impedance, flowcytometry
Langsung
Metode Rees ecker
Metode Amonium oxalat
Pemeriksaan fungsi biokimia
Pemeriksaan jalur intrinsik
activated Partial Tromboplastin Time
Pemeriksaan kelainan jalur ekstrinsik
Prothrombin time
Pemeriksaan masa pembekuan darah
metode Lee-white
Metode slide
pemeriksaan Thrombin Time (TT)
Anamnesis
Who: The patient's age, sex, racial background, and family
history of abnormal bleeding all are important.
When: Any association with a disease state, trauma, surgery, or drug ingestion should be identified. In addition, details of the time of onset and course of the bleeding event are important.
Where: The site or sites of bleeding, whether skin, mucous membranes, gastrointestinal (GI) tract, solid organ, joint, or muscle, need to be identified.
What: The physical characteristics of the bleeding, especially the distinction between petechial (capillary) hemorrhage and the purpura, ecchymoses, and hematoma formation seen with larger vessel bleeding, need to be described.
Physical examination
Examine the skin and mucous membranes (such as the inner surface of the eyelids or the mouth), looking for paleness, abnormal blood vessels (telangiectasias), bruises, small red or purple spots, or red rashes.
feel the neck, underarm, and groin areas for enlargement of lymph nodes.
Examine the joints to see if they are tender or swollen.
examine the abdomen, feeling for enlargement of the spleen or liver.
do a rectal examination to check the stool for blood.
Faktor yang dapat memperburuk perdarahan
Defisiensi Vitamin K
vitamin K sangat penting dalam koagulasi darah - perannya untuk mengaktifkan faktor-faktor pembekuan darah, menjaga kesehatan tulang, mencegah penyakit jantung sehingga defisiensi vit K dapat mengganggu proses dalam kaskade koagulasi
Melakukan kegiatan yang memperparah cedera
Pengguaan Obat-Obatan
penggunaan obat antikoagulan dan antiplatelet seperti warfarin, heparin, aspirin dapat menghambat proses pembentukan pekuan darah serta mencegah adhesi dari platelet ke subendotel situs cedera
Definisi perdarahan/gangguan perdarahan serta hemostasis
Bleeding disorders
A group of conditions in which there is a problem with the body's blood clotting process. These disorders can lead to heavy and prolonged bleeding after an injury,
Bleeding disorders fall into two main categories: inherited and acquired. Inherited bleeding disorders have a genetic predisposition and involve a deficiency of coagulation factors. Acquired bleeding disorders can be caused by conditions that an individual may develop at any point during their lifetime. These can be broader in range and dependent on comorbid conditions. The focus of this discussion will be on congenital coagulopathies; acquired bleeding disorders will be considered to be outside the scope of the information presented here.
Hemostasis
The mechanism that leads to cessation of bleeding from a blood vessel. It is a process that involves multiple interlinked steps. This cascade culminates into the formation of a "plug" that closes up the damaged sit of the blood vessel controlling the bleeding. It begins with trauma to the lining of the blood vessel
Prognosis gangguan perdarahan
Hemophilia
Prognosis baik bila diterapi dgn tepat
Prognosis gangguan perdarahan nonhemofilia tergantung pada penyebab yang mendasari. Sebagian besar gangguan perdarahan nonhemofilia adalah penyakit Von Willebrand dan prognosisnya cukup baik. Komplikasi yang paling sering adalah perdarahan masif atau perdarahan organ internal.
Gejala dari perdarahan
Gangguan perdarahan
easy bruising
bleeding gums
heavy bleeding from small cuts or dental work
unexplained nosebleeds
heavy menstrual bleeding
bleeding from joints
excessive bleeding following surgery