Tasks for understanding the topic of the lesson, methods of the type of activity

Department of Internal Diseases №1, №2.

METHODOLOGICAL INSTRUCTIONS

FOR STUDENTS

COURSE FACULTY OF MEDICINE

TO THE PRACTICE

FOR AUDITORIAL WORK

TOPIC: “Hemolytic anemia”

Approved at the cathedral meeting of the departments:

Internal Medicine No. 1,

Minutes of the meeting of the department No. 8 dated 5.02.07;

Internal Medicine No. 2,

Minutes of the meeting of the department No. 8 dated February 6, 2007;

On the methodical commission on therapy,

Minutes No. 3 dated March 7, 2007

Head Department of Internal Diseases №1

GOU VPO KrasGMA Roszdrav

MD, prof. Shulman V.A.

(signature)_________________

Head Department of Internal Diseases №2

GOU VPO KrasGMA Roszdrav

MD, prof. Tereshchenko Yu.A.

(signature)_________________

Compiled by:

PhD Assoc. Kuznetsova E.Yu.

Krasnoyarsk 2007

1. Topic of the lesson: “ Hemolytic anemia”

2 . Importance of studying the topic: Hemolytic anemia can be encountered by a doctor of any specialty. The frequency, prevalence, causes and mechanisms of development of various hemolytic anemias are not the same. The doctor’s task is to be able to timely and correctly understand the nature of hemolytic anemia and prescribe adequate treatment.

Therefore, the educational value of this topic: to have an idea about the differential diagnosis of both acquired and hereditary hemolytic anemia. The professional significance of the topic: the training of a highly qualified specialist who is well versed in the differential diagnosis of hemolytic anemia. The personal significance of the topic: the development of the responsibility of the future doctor for the differential diagnosis and adequate treatment of hemolytic anemia.

3 . Lesson objectives: on the basis of theoretical knowledge and practical skills, the student should know the pathogenesis, clinical manifestations, methods of laboratory diagnosis of hemolytic anemia, be able to assess hematological parameters in these anemias, be able to prescribe treatment, have the skills of taking an anamnesis, physical examination of patients.

4 . Topic study plan:

4.1. Independent work at the patient’s bedside – 60 min.

4.2. Practical work (analysis of patients) – 95 min.

4.3. Problem solving – 15 min.

4.4. Conclusion on the lesson (final control) in writing or orally with an assessment of knowledge – 15 min.

4.5. Task for the next lesson – 3 min.

Basic concepts and provisions of the topic

Hemolytic anemia

The main symptom of this group of anemias is shortening

lifespan of erythrocytes. Hemolysis can occur in a variety of pathological processes, occur continuously or episodically (in the form of crises), be predominantly intracellular or intravascular.

There are hereditary and acquired hemolytic anemias.

Hereditary microspherocytosis (Minkowski-Chaffard disease) is diagnosed in the presence of normochromic microspherocytic anemia, signs of hemolysis, hereditary stigmas, an autosomal dominant type of inheritance of reduced osmotic resistance of erythrocytes, a combination of hemolytic anemia, cholelithiasis at a young age.

Sickle cell anemia is diagnosed by examining erythrocytes in a light microscope, carrying out hemoglobin electrophoresis.

Hemolytic anemia associated with deficiencies in erythrocytes of glucose-six-phosphate dehydrogenase is diagnosed by examining the content of this enzyme in erythrocytes.

Autoimmune hemolytic anemia is a fairly common disease in which autoantibodies to one’s own erythrocytes are formed.

These anemias can be independent or accompany other diseases (chronic hepatitis, liver cirrhosis, systemic diseases, lymphoproliferative tumors, etc.).

Idelpathic AIHA are classified according to the type of antibody:

AIHA with incomplete heat agglutinins

AIHA with thermal hemolysins

AIHA with complete cold agglutinins

AIHA with total cold hemolysins

Common signs of hemolytic anemia are:

normochromic anemia

Erythrocytes may show signs of mechanical damage (schizocytes)

High reticulocytosis

Increasing the content of indirect bilirubin

Increasing the content of serum iron in intracellular hemolysis

Iron deficiency in intravascular hemolysis

Enlargement of the spleen with intracellular hemolysis

Nontrophilic leukocytosis in crises

Positive Coombs test for autoimmune anemia

Isolation of black urine, the appearance of free hemoglobin in plasma, hemosedrin in the urine with intravascular hemolysis

Hyperplasia of an erythroid germ in the c / brain

Treatment of hemolytic anemia:

RBC replacement therapy only

strict indications after typing of erythrocytes of the donor and recipient according to the indirect Coombs test or washed erythrocytes 4 times in saline.

Hormone therapy (1-2 mg of prednisolone per 1 kg of the patient’s weight)

Splenectomy with frequent crises (2-3 relapses per year)

Cyclophosphamide 200-400 mg every other day IV, course dose 6-8 g and other immunosuppressants.

Plasmapheresis.

Thalassemia

Hereditary hemolytic anemia caused by impaired synthesis of globin chains.

There is a deletion of the gene responsible for the synthesis of globin, which leads to structural disorders in hemoglobin, impaired iron assimilation during hemoglobin synthesis and, simultaneously, to early death of erythrocytes.

Disrupted synthesis of one of the globin chains leads to an imbalance of chains (which exist normally), a chain that is produced in excess, aggregates and is deposited in erythrokaryocytes – the reason for ineffective erythropoiesis.

The most common is B-thalassemia. In the heterozygous form, the content of fractional hemoglobin A2 and fetal hemoglobin slightly increases. In the homozygous form, mainly fetal hemoglobin increases, hemoglobin A2 also increases, but in a larger percentage. It is established by electrophoresis of hemoglobin.

The main signs of thalassemia:

Anemia hypochromic

Reticulocytosis

Targeting of erythrocytes, their basophilic puncture.

The development of anemia in childhood, its hereditary nature.

Increase in indirect bilirubin

An increase in the content of iron in the blood serum.

Enlargement of the spleen

The diagnosis is verified by hemoglobin electrophoresis.

Treatment – splenectomy, desferal for life.

Tasks for understanding the topic of the lesson, methods of the type of activity

Test control:

1. Thalassemia is based on:

A) violation of the synthesis of porphyrins

B) violation of the synthesis of globin chains

C) deficiency of G-6-FDG

D) iron deficiency

2. What kind of hemolytic anemia occurs with erythrocyte hypochromidia and a decrease in color index:

A) Minkowski-Shafar disease

B) thalassemia

B) anemia with hypersplenism

D) autoimmune hemolytic anemia

D) Marknafava-Micheli disease

3. Symptoms of diagnostic value in hemolytic anemia:

A) the degree of decrease in the amount of hemoglobin and the number of erythrocytes

B) reticulocytosis

B) splenomegaly

D) the level of indirect bilirubin

D) bone marrow punctate

4. Among hemolytic anemias, there are:

A) hereditary

B) Acquired

B) symptomatic

D) Idiopathic

D) All of the above

5. Hereditary hemolytic anemias caused by a defect in the erythrocyte membrane include:

A) Minkowski-Schoffard disease

B) aplastic anemia

B) thalassemia

D) Marchiafava-Micheli disease

6. The defect in hereditary microspherocytosis is localized in:

A) The protein structure of the erythrocyte membrane

B) Lipids of the erythrocyte membrane

B) The structure of the heme

D) Globin chains

D) Correct C and D

7. The diagnosis of Minkowski-Choffard disease is based on all of the listed studies, except for:

A) Morphology of erythrocytes

B) Increasing the level of indirect bilirubin

B) Coombs’ tests

D) Osmotic resistance of erythrocytes

8. Hemolytic anemias are characterized by:

A) Jaundice of the skin and sclera

B) Petechiae on the skin

B) splenomegaly

D) Lymphadenopathy

E) Rule A and B

9. Diagnostic criteria for thalassemia are:

A) hyperchromic anemia

B) Low iron content in blood serum

C) Decreased osmotic resistance of erythrocytes

D) Sharp irritation of the red germ in the myelogram

10. Thalassemia treatment does not include:

A) The appointment of cytostatics

B) Transfusion of erythrocytes

B) splenectomy

D) bone marrow transplant

11. Most often, sickle cell anemia occurs in all of the following countries except:

A) Azerbaijan

B) Georgia

B) Central Africa

D) Scandinavian countries

12. The following clinical findings are typical for sickle cell anemia:

A) Hyperchromia of erythrocytes

B) Reticulocytopenia

C) An increase in the fraction of indirect bilirubin

D) Changes in hemoglobin electrophoresis

1. Situational tasks: 1. In which of the following situations is transfusion of erythrocytes indicated when hemoglobin drops to 70 g/l?

BUT). A 35-year-old patient with iron deficiency anemia against the background of menorrhagia with signs of hemosiderosis.

B). A 65-year-old patient with B12 deficiency anemia, coronary artery disease, postinfarction cardiosclerosis (RR 18 per minute, BP 130/80 mm Hg).

AT). A 62-year-old patient with hereditary microspherocytosis, cholelithiasis, ischemic heart disease, atherosclerotic cardiosclerosis, atrial fibrillation and signs of heart failure.

G). A 45-year-old patient with alcoholic cirrhosis of the liver with portal hypertension and splenomegaly.

D). A 40-year-old patient with folic deficiency anemia on the background of chronic alcoholism with chronic alcoholic pancreatitis, alcoholic polyneuritis.

2. An 18-year-old girl has slight jaundice and a slight enlargement of the spleen. In the analysis, normochromic anemia with reticulocytosis 120%. The patient’s brother was operated on for gallstones at the age of 26. What research, along with a thorough study of the morphology of erythrocytes, will confirm your diagnosis?

A) The content of vitamin B12 and folic acid.

B) Electrophoresis of hemoglobin.

C) Serum protein electrophoresis.

D) Serum iron.

D) Osmatic and acid resistance of erythrocytes.

3. A patient suffering from chronic lymphocytic leukemia of a benign course (leukocytes 18*109/l, lymphocytes 80%, platelets 220*109/l) has severe normochromic anemia. The spleen is slightly enlarged. The most likely cause of this anemia is:

A) Suppression of erythropoiesis by a leukemic cell clone.

B) autoimmune.

B) hypersplenic.

D) Folic deficiency.

4. A 6-year-old child has pale skin with an icteric tint, an enlarged spleen 4 cm below the costal arch. In the blood test: Hb 82g/l, erythr. 2.1, c.p. 0.9, reticulocytes 175‰, indirect bilirubin 38 µmol/l. Preliminary diagnosis: hereditary microspherocytosis (Minkowski-Choffard disease)

1. What should be determined to confirm the diagnosis?

2. What is the main cause of anemia?

3. Treatment tactics?

4. What complication is possible?

5. A 34-year-old patient complains of fatigue, dizziness, periodic paroxysmal pain in the right hypochondrium. Such complaints have been disturbing since childhood, during exacerbations the skin and sclera are icteric. Heart sounds are sonorous, rhythmic, systolic murmur at the top. The abdomen is soft, painful in the right hypochondrium and at the point of the gallbladder. Liver at the edge of the costal arch, dense, slightly painful, spleen +5 cm, dense. In the blood test: Hb 80g/l, erythr. 2.4, c.p. 1.0, lake. 9.8, thrombus. 150.0, reticulocytes 140‰, ESR 30mm/h. Coombs reaction is negative, indirect bilirubin is 36 µmol/l.

1. Hematological characteristics of anemia?

2. What is your diagnosis?

3. What is expected during an ultrasound of the gallbladder?

4. What is the osmotic resistance of erythrocytes?

5. Primary treatment?

6. A 56-year-old patient notes an increase in general weakness, shortness of breath when walking, dizziness, fainting. For 10 days she took biseptol for exacerbation of chronic pyelonephritis. On examination, the skin is pale with subicteric. Lymph nodes are not enlarged. The liver and spleen are not palpated. In the blood test: Hb 78g/l, erythr. 2.4, c.p. 0.92, reticulocytes 180‰, leuk. 13.5, thrombus. 189, ESR No.:mm/h.

1. Characteristics of the hemogram

2. Possible cause of anemia?

3. What tests are needed to confirm the diagnosis

4. Your appointments

7. A 30-year-old patient, 3 days after vaccination against influenza, had a fever up to 38C, general weakness, shortness of breath at rest, red urine began to increase. The spleen is not palpable. In the blood test: Hb 60g/l, erythr. 1.9, c.p. 1.0, reticulocytes 278‰. free hemoglobin was found in plasma. In the analysis of urine a large amount of hemosiderin.

1. What is the nature of anemia in a patient?

2. Differential diagnosis?

3. What examinations should be carried out?

7. List of topics on UIRS, proposed by the department:

1. Create an algorithm for the differential diagnosis of hereditary hemolytic anemia 2. Write an abstract: Diagnosis and treatment of thalassemia 3. Write an abstract: Diagnosis and management of anemia associated with dedicitis glucose 6 phosphate dehydrogenase. 4. Write an abstract: Diagnosis and management of a patient with sickle cell anemia. 5. Create an algorithm for the differential diagnosis of acquired hemolytic anemia.

8. Recommended reading:

Main:

1. Internal diseases: textbook: in 2 volumes / ed. ON THE. Mukhina, V.S., V.S. Moiseeva, A.I. Martynov. – 2nd ed. correct and additional – M.: GEOTAR – Media, 2006.

2. Internal diseases according to Tinsley R. Harrison: In 2 books / Ed. E. Fauci and others – M .: Practice, 2002. – (Classics of modern medicine) – Book 1. – 2002. – 1536 p. – Book 2.-2002.-1760 p. Additional:

1. Guseva, S. A. Diseases of the blood system: a Handbook/S. A. Guseva, V. P. Voznyuk.-M.: MEDpress-inform, 2004.-488 p.

2. Kotoyan, E.R. Clinical Hematology: A Guide for Physicians.-M.: Med. inform. agency”, 2003.- 245 p.

3. Novik A.A., Anemia (from A to Z): A guide for doctors / A.A. Novik, A.N. Bogdanov.-SPb.: Neva, 2004.- 320 p.

Teaching aids:

1. L.N. Timofeeva, V.Ya. Romanova. Clinical syndromes in hematology. Uch-met. allowance Approved. TsKMS KrasGMA Krasnoyarsk. LLC “Verso” -2006.

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