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I. Importance of the Subject




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I. Importance of the Subject.

The definition of “a hemorrhagic diathesis” comprises a group of various diseases and syndromes whose distinctive feature is an increased bleeding. It can be caused by different pathogenetic mechanisms. The first group deals with the diseases related to changes in the number and functions of thrombocytes (thrombocytopenia and thrombocytopathy). The second group comprises diseases the bleeding of which is caused by blood coagulation disorders as a result of a hereditary or acquired deficiency of procoagulants or increased content of anticoagulants (hemophilia, dysprothrombinemia, hypo- and afibrinogenemia). The third group comprises diseases the bleeding of which is caused by damage to the vascular wall (Schonlein-Henoch disease, Rendu-Osler disease, etc.). From the above, it may be deduced that there is a clinical diversity of hemorrhagic diathesis manifestations. Only knowledge of pathogenesis of a certain disease allows to select the only correct way of patient management.

The patients affected by a hemorrhagic diathesis are met with in the medical practice of a hematologist, a therapeutist, a pediatrician, a surgeon, a traumatologist, a gynecologist, a dentist and doctors of other specialties.


If the importance of the subject is clear, then the students shall proceed to learning the Practice objectives.


^ II. Learning Objectives.

Key objective: To develop skills in making a differential diagnosis of different types of bleeding and diseases accompanied by a hemorrhagic syndrome, planning a patient management and providing the first aid in emergency cases.


^ Specific Objectives – To gain knowledge of and develop skills in:

1. Identifying the syndromes of pathology of the hemostasis system.


Pre-practice knowledge and skills:


1. Selecting the information representative of hemostasis diseases from the complaints, case history and physical examination.

2. Setting up a diagram of an individual diagnostic search and interpret these findings in case of a hemorrhagic syndrome.


3. Making a differential diagnosis of different diseases caused by the hemostasis system disorders.

4. Diagnosing urgent conditions in case of various hemorrhagic diathesis.


5. Justifying the patient management principles and providing medical aid in case of emergency for patients with hemorrhagic diathesis.


2. Determining the necessary amount and sequence of investigative methods:

  • laboratory: a general blood count, a coagulogram and a hemophilia type test. (Explained in the Physiology and Pathophysiology Course).

  • Instrumental: X-ray of the joints, fiberoptic gastro-duodenoscopy, ultrasonic scanning of the organs of the abdominal cavity. (Explained in the X-ray Diagnostics Course).

3. Distinguishing syndromes in case of hemorrhagic diathesis. (Explained in the Propaedeutics and Therapy Course).

4. Distinguishing syndromes in case of emergency in the patients affected by hemorrhagic diathesis (Explained in the Propaedeutics and Therapy Course).

5. Applying the medications of pathogenetic and symptomatic therapy. (Explained in the Pharmacology Course)


The information necessary for reviewing and revising the basic knowledge and skills can be found in the following references:


  1. Arthur C. Guyton, John E. Textbook of Medical Physiology (Textbook Binding). - Hall W.B. Saunders Company; 10th edition. – 2000.

  2. Aaron B. Caughey, Nancy Palmer, Christie Del Castillo, and Dana Tuttle. Pathophysiology: Hematology and Oncology. -Blueprints Notes & Cases Series. - 2003.

  3. Vinay Kumar, Nelso Fausto, Abul Abbas. Pathologic Basis of Disease. - Robbins & Cotran, Seventh Edition. – 2004.

  4. Carol Porth. Pathophysiology.- Lippincott Williams & Wilkins. – 2004.


Initial Test Questions.

Q1. During Konchalovsky-Rumpel-Leede cuff test modified by Borchgrevik, the following results were obtained for a 23-year-old patient: the petechia number in an outlined circle was 53.

Which of the following can be the correct result of this test in the patient?

  1. no appreciable disease;

  2. low positive test;

  3. positive test;

  4. sharply positive test;

  5. negative test.


Q2. A blood count of a 38-year-old woman presents the following evidence: erythrocytes 4,05×1012, Hb is 138 g/l, thrombocytes-120х109, leukocytes 4.1х109, 5 band neutrophils, 55 segmented neutrophils, 35 lymphocytes, 4 monocytes and ESR is 10 mm/h.

Which of the following is a correct name for the changes?

  1. NAD (no appreciable disease);

  2. thrombocytosis;

  3. normochromal anemia;

  4. Leukopenia;

  5. thrombocytopenia.


Q3. During an examination of the skin of a 45-year-old patient, small dotted and spotted painless hemorrhages were revealed that, according to the patient occured spontaneously.

Which of the following types of bleeding is present in the patient?

  1. petechially-spotty;

  2. hematic;

  3. vasculitis-purpuric;

  4. angiomatous;

  5. mixed.


Q4. A physical examination of a 40-year-old patient revealed hematoma of the right knee joint. Taking a case history revealed that the patient's uncle had been suffering from hemophilia B.

Which of the following parts of the hemostasis system was affected in the patient?

  1. vascular;

  2. plasmatic;

  3. thrombocyte-thrombocytopenia;

  4. thrombocyte-thrombocytopathy;

  5. all of the above.


Q5. The results of a laboratory investigation of a 25-year-old patient having a record of coagulopathy, in his case history showed the following: bleeding time was 8 min, and venous blood coagulation time was 10 min.

Which of the following is a correct explanation?

  1. both indices are within the normal range.

  2. bleeding time is within the normal range, but blood coagulation time is increased;

  3. bleeding time is increased, but blood coagulation time is within the normal range;

  4. both indices are higher than normal;

  5. both indices are lower than normal;


III. Theory Topics on the Subject


To reach the learning objectives it is essential to master the following theory issues:

  1. Complaints, peculiarities of the case history, objective data of the patients affected by hemorrhagic diatheses.

  2. Methods of making a clinical and laboratory diagnosis of hemorrhagic diathesis.

  3. Making a differential diagnosis of hemorrhagic diatheses.

  4. Classification of hemorrhagic diatheses.

  5. Etiology and Pathogenesis of Hemorrhagic Diatheses.

  6. Principles of treatment of hemorrhagic diatheses and methods of providing a medical aid in case of emergency related to a hemorrhagic syndrome.


The information necessary for mastering the theory issues may be found in the following references:

  1. Alan Rosenwald. Practical Diagnosis of Hematologic Disorders, Fourth Edition. – 2006. - P.168-197.

  2. Chris Cheson Clinical Laboratory Hematology.- Delphi Publishers. – 2007. –P. 54-109.

  3. Douglas Williams. Hematology, Seventh Edition. - Williams Hematology. – 2006. – P. 285-302.

  4. Clinical Hematology Atlas. - Robbins & Cotran. – 2007.

  5. Jeremi Wilks. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. – Stella’s Production. – 2006.

  6. Lecture notes on hospital therapy.

  7. Flow Chart of "Hemorrhagic Diatheses»(Appendix 1)

  8. Diagnostic Algorithm of a Hemorrhagic Syndrome. (Appendix 2).

  9. Classification of hemorrhagic diatheses (Appendix 3).

  10. Types of bleeding (Appendix 4).


Then, proceed to answering some test questions and checking your answer for question 5 by the key.


Q1. A severe nasal bleeding started in a 32-year-old patient affected by hemophilia A. Which of the following drugs was prescribed to this patient?

  1. vicasol;

  2. thromboconcentrate;

  3. cryoprecipitate;

  4. prednisolone;

  5. etamsylate.


Q2. A 20-year-old patient was delivered to the hematology department complaining of a hemorrhagic rash on the skin of his lower and upper extremities. A physical examination detected symmetrical hemorrhagic papulae in places with urticaria and single central necroses covered with crust. If pressed upon with a microscope slide, they do not disappear. Which of the following is a preliminary diagnosis?

  1. Rendu-Osler disease;

  2. hemophilia C;

  3. hemorrhagic vasculitis, skin type;

  4. idiopathic thrombocytopenic purpura;

  5. thrombocytopathy;


Q3. A 34-year-old patient affected by Rendu-Osler disease often experiences nausea, and then vomiting of the color of coffee grounds. Fiberoptic gastroduadenoscopy detected multiple bleeding telangiectasias. Which of the following is a correct patient management?

  1. introduction of dicynon;

  2. introduction of vicasol;

  3. introduction of aminocaproic acid;

  4. introduction of thromboconcentrate;

  5. surgical methods of treatment, such as excision of the areas rich in telangiectasias.


Q4. An 18-year-old patient was delivered to an ENT department by ambulance due to an incessant nasal bleeding that started after an injury. Introduction of dicynon, application of a hemostatic sponge and nasal packing were to no avail. In case of rhinoscopy, the otolaryngologist noticed multiple telangiectasias on the mucous membrane in the nasal cavity. Which of the following diseases can be assumed?

  1. purpura rheumatica;

  2. autoimmune idiopathic thrombocytopenia;

  3. hemophilia A;

  4. congenital telangiectasia (Rendu-Osler disease);

  5. Wegener's granulomatosis.


Q5. A 32-year-old patient affected by idiopathic thrombocytopenic purpura developed uterine bleeding.

Which of the following methods of the emergency medical aid must be administered to the patient?

  1. introduction of vicasol;

  2. hysterectomy;

  3. introduction of calcium chloride;

  4. infusion of thromboconcentrate;

  5. introduction of aminocaproic acid.


Teaching Guidelines for Practice Activities on "Hemorrhagic diathesis".

At the beginning of the class, the instructor gives a test to check and correct the pre-practice level of knowledge. Then, the students start to see patients under the instructor’s supervision after which the students give an analysis of the practice patients and the instructor checks the mastering of the practice topics. A final test is provided to strengthen the knowledge gained. At the end of the class, the instructor sums up the students' self-learning activity.

^ F
Appendix 1

Hemorrhagic diatheses
low Chart of "Hemorrhagic Diatheses”



Complaints of spots occurred on the mucous membrane and on the skin of petechiae; gingival, nasal and gastrointestinal hemorrhges; occurence of hematomas, presence of vascular "stars» and rash along the vessels.

Objective examination showed: discovery of elements of the hemorrhagic rash at different stages of development, hemarthroses, vascular "spiders", "little spiders", papular symmetric skin rash along the vessels and signs of hemorrha-ges of various degrees of manifestation.






Case history





^


History of burdened heredity and triggering factors





Hemorrhagic syndrome



Additional investigation findings





Instrumental


Laboratory


General blood count (reduced number of thrombocytes, decrease in the blood clot retraction, prolonged bleeding time, prolonged blood clotting time as per Lee-White, general urine analysis (hematuria), increased level of CIC, myelogram (increased number of megacaryocytes, bone marrow irritation, and decreased number of thrombocytes.

X-ray of joints: narrowing of the joint space, ankyloseness, rhinoscopy (discovery of vascular spiders on the mucous membrane of the nasal cavity), fiberoptic gastroduadenoscopy shows the same changes on the mucous membrane of the esophagus and stomach), ultrasonic scanning of the liver and spleen (enlargement and structural irregularity).






Differential diagnosis by a diagnostic algorithm: idiopathic thrombocytopenic purpura. Hemophilia. Rendu-Osler disease. Purpura rheumatica.






Patient management






Pathogenetic therapy corticosteroids, cytostatic agents, cryoprecipitate, fresh frozen plasma, anticoagulants and cryoablation of telangiectasias.

Symptomatic therapy: intravenously or by mouth: dicynon, ascorutin, aminocaproic acid, and fresh frozen plasma, locally: hemostatic sponge, thick bandage or tamponade and ice.



Appendix 2.

Diagnostic Algorithm of "Hemorrhagic Diatheses".

  1. List of Nosological Units.

  2. Idiopathic thrombocytopenic purpura (Werlhof's disease).

  3. Hemophilia A, B, C.

  4. Congenital telangiectasia (Rendu-Osler disease).

  5. Purpura rheumatica (Schonlein-Henoch disease).









Hemorrhagic syndrome


yes


Hemorrhagic elements: punctulated, spotted, irregular, painless, nasal, gingival hemorrhages and the decreased number of thrombocytes in a general blood count.

Hematomas, hemarthroses, delayed hemorrhages (a few hours later after an injury), and the number of thrombocytes in the peripheral blood is within the normal range


no







1

yes

no



2

Telangiectasias look like "stars", "spiders»and are on the skin and visible mucous membranes disappearing when pressed with a specimen slide and all these changes are observed since birth.


3

yes

Papular- urticarial hemorrhagic elements, symmetric, along the vessels on the extremities, necrosis is likely to occur in the center of te elements, the articular syndrome and macro- or microhematuria

no

4

yes

no

5

no

Appendix 3.

Classification of hemorrhagic diatheses.

  1. Diseases related to the changes in the number and functions of thrombocytes (thrombocytopenia and thrombocytopathy).

  2. Diseases the bleeding of which is caused by blood coagulation disorders as a result of a hereditary or acquired deficiency of procoagulants or increased content of anticoagulants (hemophilia, dysprothrombinemia, hypo- and afibrinogenemia).

  3. Diseases the bleeding of which is caused by damage to the vascular wall (Schonlein-Henoch disease, Rendu-Osler disease, etc.).


Appendix 4.

Types of bleeding

  1. Hematic – which is with sore tense hemorrhages both into the soft tissues and into joints, evident pathology of the locomotor system which is typical for hemophilia A and B;

  2. petechial-spotty (bruise-like) is typical for thrombocytopenias, thrombocytopathies and some disorders of blood coagulability - hypo- and dysfibrinogenemias, hereditary deficiency of factor X and ІІ, sometimes VII.

  3. mixed bruise-like-hematic type characterized by a combination of petechial-spotty hemorrhage with occurrence of single large hematomas (retroperitoneal ones, in the intestinal wall, etc.) in case of absence of any damage to the joints and bones (difference from the hematic type). This type of bleeding is observed in case of a severe deficiency of the factors of the prothrombin complex and factor XIII, Willebrand's disease, disseminated intravascular coagulation (DIC) syndrome, overdose of anticoagulants and thrombolytic drugs, in case of occurrence of immune inhibitors, such as factor VIII or IX in the blood;

  4. vasculitis-purpuric type is characterized by hemorrhages in the form of rash or erythema (caused by inflammation), nephritis and intestinal bleeding are possible, it is observed in case of infectious and immune vasculitis, it is easily transformed into the DIC-syndrome;

  5. an angiomatous type is observed in case of telangiectasia, angioma arterial-venous shunts and is characterized by persistent strictly located and attached to the local vascular pathology with hemorrhages.

Final Test Questions

Q1.

A 35-year-old patient was first delivered to the hematology department complaining of spontaneously occurring hemorrhages on the upper and lower extremities being painful if pressed upon. The blood count presents the following evidence: erythrocytes are 3.5х1012, Нb-120 g/l, color index is 0.9, thrombocytes are 85×109, leukocytes are 5.0×109, 5 band neutrophils, 50 segmented neutrophils, 35 lymphocytes, 10 monocytes and ESR is 15 mm/h.

Which of the following is the most likely diagnosis?

  1. hemophilia A;

  2. purpura rheumatica;

  3. Rendu-Osler disease;

  4. idiopathic thrombocytopenic purpura;

  5. aplastic anemia.


Q2.

A 34-year-old patient affected by purpura rheumatica in its cutaneous-articular form, had protein in his urine of 2.5 g/l during his regular exacerbation of the disease, including cylindruria and microhematuria.

Which of the following medications has been prescribed to this patient?

  1. prednisolone;

  2. vicasol;

  3. aminocaproic acid;

  4. sodium etamsylate;

  5. aspirin.


Q3.

A 22-year-old patient was delivered to the hematology department by ambulance due to a severe bleeding from the alveolar socket after it had been extracted. The bleeding stared 2 hours later after seeing the dentist. A talk to the patient turned out that his brother was affected by hemophilia A, but the patient had never shown any signs of the disease before.

Which of the following is the most likely diagnosis?

  1. Autoimmune idiopathic thrombocytopenia;

  2. Hemophilia A;

  3. Hemophilia B;

  4. Hemophilia C;

  5. Rendu-Osler disease


Q4.

A 20-year-old patient affected by hemophilia B had signs of hemarthrosis of the left knee joint.

Which of the following drugs must be prescribed to this patient?

  1. cryoprecipitate;

  2. thromboconcentrate;

  3. fresh frozen plasma;

  4. indometacin;

  5. aminocaproic acid.


Q5.

A 38-year-old patient affected by autoimmune thrombocytopenia underwent splenectomy due to the absence of any therapeutic effect from a glucocorticoid therapy. However, the patient's condition did not change: the hemorrhagic syndrome remains, the number of thrombocytes in the peripheral blood did not increase as compared to the initial one.

Which of the following drugs must be added to the therapeutic regimen of this patient?

  1. dicynon;

  2. vicasol;

  3. azathioprine;

  4. Fresh frozen plasma;

  5. Calcium chloride.




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