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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3256_Библиотеки_им_академика_М_И_Перельмана
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performing immunophenotyping, it is recommended to evaluate the
expression of CD38, ZAP-70 and CD52. Expression of CD38 and /
or ZAP-70 correlates with the detection of “u-CLL” and is a poor
prognostic sign. The CD52 marker is a “target” for treatment with
a monoclonal anti-CD52 antibody (Campath-1).
¾ Cytogenetic and molecular genetic studies in chronic
lymphocytic leukemia. In a routine cytogenetic study, changes in the
karyotype are found in more than 50% of patients with CLL, with
FISH analysis - in 80%. The most common abnormalities include
trisomy 12 (20% of cases), deletion of chromosomes llq (20%), 13q
and 14q (50%), 6q21 (5%), or 17p13 (locus p53 - 10% of cases). With
changes in the karyotype, especially multiple changes, the prognosis
of the disease worsens.
3. What is the patient’s management?
There are several approaches to the treatment of chronic lympho-
cytic leukemia:
y Chemotherapy with chlorbutin was until recently the standard
treatment for chronic lymphocytic leukemia. Currently, the use
of a new group of drugs, the so-called purine analogs, of which
Fludara is a representative, has been proven to be more eective.
y Eective methods of treating chronic lymphocytic leukemia
include bioimmunotherapy using monoclonal antibodies. The
introduction of these drugs allows you to selectively destroy tumor
cells without damaging healthy tissues of the body.
y If the eectiveness of other treatments is unsatisfactory, high-dose
chemotherapy with hematopoietic stem cell transplantation can be
carried out.
y Radiation therapy is used as an adjunct treatment in the presence
of a large tumor mass.
y “Removal of the spleen (splenectomy) is sometimes indicated
when the organ is greatly enlarged.
4. Medical and labor expertise. Dispensary observation.
Indications for medical and social expertise.
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y The need for rational employment of patients with chronic
lymphocytic leukemia, stage 1;
y II, III, IV stages of the disease;
y transformation of chronic lymphocytic leukemia into
a malignant form;
y progression of chronic lymphocytic leukemia or its complications.
The required minimum examination for medical and social ex-
pertise: blood test, platelets and reticulocytes, myelogram, Immuno-
phenotyping of blood lymphocytes, total protein and fractions, chest
x-ray, ultrasound of the abdominal organs, consultation with an oph-
thalmologist and neurologist, a hematologist’s conclusion.
Disability criteria.
III group of disability is established for patients with I, II stages
of the disease, in the absence of its progression, limitation of the abil-
ity to work, I st., with the aim of rational employment, retraining or
reducing the amount of work performed.
II group of disability is established for patients with III-VI stage
of the disease, requiring specic treatment, due to severe dysfunc-
tions, limitation of the ability to self-service, independent movement
of the 2nd degree, labor activity of the 2nd degree.
Labor is available only in specially created conditions.
Group I disability is established for patients with stage IV disease
in the presence of severe anemia and thrombocytopenia, persistent se-
vere infection and complications; with the rapid progression of the
disease, transformation into a malignant form, limitation of the ability
to self-service 3 tbsp., independent movement of 3 tbsp., and an unfa-
vorable prognosis.
Dispensary observation. Patients with a conrmed diagnosis of
CLL are under dynamic observation by a hematologist-oncologist.
A blood test is performed every 1-3-6 months. If necessary, supportive
cytostatic therapy is prescribed to contain leukemic aggression.
Prophylaxis
To date, there is no specic prophylaxis for chronic lymphocyt-
ic leukemia.
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373
Treatment with herbs and other non-traditional folk methods is
futile, and in some cases it is deadly for the patient.
Clinical case No. 3
1. What is the preliminary diagnosis? Chronic post hemorrhag-
ic iron deciency anemia of grade 3.
2. What additional tests would you recommend to the patient,
and what are the expected results?
¾ Study of serum iron: the norm for men is 0.6–1.7 mg / l (13–
30 µmol / l); the norm for women is 0.5–1.6 mg / l (12–25 µmol / l).
With IDA, serum iron is reduced. Determination of serum ferritin is
the most sensitive and specic laboratory sign of iron deciency: the
norm for men is 20–350 μg / l; the norm for women is 10–150 mcg / l.
With IDA, a decrease in serum ferritin levels.
¾ Total serum iron-binding capacity (TIBC) reects the degree
of serum starvation. The norm is 30–85 μmol / l. With IDA, the value
of the indicator increases. The latent iron-binding capacity of serum
is on average 50.2 μmol. With IDA, the indicator increases. The last
two tests are rarely used in clinical practice. The ratio of the iron index
to the total iron-binding capacity of serum, expressed as a percentage,
reects the degree of saturation of transferrin with iron (the norm is
16-50%). The IDA is characterized by a decrease in this indicator.
Desferal test (desferal complexone, which binds iron and excretes
it in the urine). After intravenous administration of desferal in the
urine, 0.8 to 1.2 mg of iron is normally excreted. With IDA - 0.2 mg
and below.
¾ In the punctate of the bone marrow, as a rule, without
signicant changes: some irritation of the red sprout (with bleeding);
a decrease in the number of sideroblasts; determining the cause of
iron deciency.
Instrumental research
According to indications, ECG, ultrasound of internal organs,
EGDS / colonoscopy are performed.
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374
3. What is the patient’s management?
IDA treatment in women of reproductive age
1. Women of reproductive age with moderate (mild or moderate)
IDA should receive iron supplements at the rate of 120 mg of elemen-
tal iron per day (60 mg x 2 times a day) for 3 months.
2. For women of reproductive age with severe IDA, iron prepara-
tions are prescribed at the rate of 180 mg of elemental iron per day (60
mg x 3 times a day) for 3 months.
3. Folic acid for women of reproductive age with IDA is pre-
scribed in a dose of 0.4 mg daily for 3 months.
4. Iron preparations are best taken between meals, it is advisable
to drink it with juice or boiled water. It is forbidden to drink tea, cof-
fee.
5. Evaluation of adherence to therapy is carried out 14 days after
the start of treatment.
6. 4 weeks after the initiation of IDA treatment, Hb should be
re-determined. If there is a positive result on the prescribed treat-
ment (any increase in Hb compared to the baseline level before the
start of treatment), then the treatment should be continued for another
2 months. The general course of treatment with iron is 3 months, fol-
lowed by a transition to a prophylactic regimen of weekly supplemen-
tation (see above).
7. In the absence of a positive result from the treatment (an in-
crease in hemoglobin compared with the baseline values
before the
appointment of iron preparations), the doctor should determine the
possible reasons for the lack of improvement: an insucient dose of
elemental iron, irregular intake or refusal to take iron supplements.
If errors in treatment are excluded, the woman should be referred to
a therapist or hematologist (in TB, OB) to clarify the form of anemia.
8. Women with severe anemia (Hb below 70 g / l) should be con-
sulted by a physician or hematologist.
9. In severely emaciated women, anemia is often severe. How-
ever, oral iron therapy should not be started until the woman’s appetite
is restored and she begins to gain weight. If appetite or weight is not
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375
normalized, the patient should be referred to a hematologist for further
examination.
4. Medical and labor expertise. Dispensary observation.
Temporary disability is due to both the anemia itself and the
disease that caused it. With a mild form of anemia (hemoglobin at
least 90 g / l), work capacity is determined by the course of the un-
derlying disease. Patients are usually able to work. With a moderate
degree, temporary disability is approximately 14–18 days; in severe
cases - 30–35 days. Contraindicated types and working conditions:
hard physical labor; work associated with neuropsychic stress, inso-
lation, exposure to various radiation sources, hematological poisons
(lead, chlorine, etc.), general and local vibration, as well as staying at
a height, servicing moving mechanisms.
Permanent disability
Disability of the III group is established by patients with IDA of
moderate severity due to the limitation of the ability to work, self-
service - I st.
Disability of group II is established in patients with IDA of se-
vere course with the development of severe complications of the cir-
culatory system, other organs, ineectiveness of treatment, leading to
a limitation of the ability to self-service, movement, learning, work - II
Art. In some cases, patients can work in specially created conditions
or at home.
Dispensary observation. Persons with latent iron deciency are
not subject to medical examination. All patients with IDA, as well as
persons with risk factors for this pathology, must be registered with
a family doctor at the polyclinic at the place of residence with the
obligatory conduct of a general blood test at least 2 times a year and
a study of the serum iron content. At the same time, dispensary ob-
servation is also carried out, taking into account the etiology of IDA,
that is, the patient is registered at the dispensary for the disease that
caused IDA.
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Clinical case No. 4
1. What is the preliminary diagnosis?
Acute leukemia
2. What additional tests would you recommend to the patient,
and what are the expected results?
¾ The bone marrow is punctured with a needle inserted into the
bone cavity. Usually the chest is selected for biopsy. The study shows
the presence of an acute or chronic process. The analysis can determine
the cytogenetic and morphological type of the disease. This procedure
checks the response of the aected cell to the chemotherapy drug.
¾ A myelogram reveals the ratio of abnormal cells to normal
cells. The examination shows the degree of damage to the organism.
In a healthy person, blast cells should not exceed 5%. The test material
is represented by a bone marrow sample. During the procedure,
lymphocytosis and the nature of atypical cells are detected. There are
no megakaryocytes. This method is recognized as the most accurate
for making a diagnosis.
¾ Cytochemical laboratory diagnostics is based on the
determination of specic enzymes. The method is indispensable for
determining acute forms of the disease and predicting their course.
¾ Immunophenotyping is performed to diagnose acute
lymphocytic and myeloid leukemia. The tactics of treatment of these
types of leukemia are dierent, so it is important to clarify the diagnosis.
¾ It is necessary to examine the cerebrospinal uid (cerebrospinal
uid) to detect an increased number of cells (cytosis).
Instrumental methods. With the help of computed tomography,
vascular damage and metastases in the lymph nodes are detected. The
method is used for large-scale diagnostics of the body by means of
layer-by-layer scanning. Chest X-ray is prescribed for characteristic
symptoms of lung damage: regular coughing, sputum production with
blood inclusions, chest pain. If the oncological process has aected
the brain, magnetic resonance imaging is recommended. The disease
is characterized by symptoms: blurred vision, numbness of the limbs,
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377
impaired memory and consciousness, dizziness and fainting. To detect
abnormalities in the work of the heart muscle, an electrocardiogram
and an echocardiogram are performed.
3. What is the patient’s management?
Depending on the type of leukemia, polychemotherapy courses
are carried out (for acute myeloid leukemia - according to the 7 +
3 program - cytosar, rubomycin, for acute lymphoblastic leukemia -
a two-phase eight-week scheme according to D. Hollzer.) Against the
background of thromboconcentrate transfusions, antibiotic therapy
4. Medical and labor expertise. Dispensary observation.
III group of disability is established for patients with I, II stages
of the disease, in the absence of its progression, limitation of the abil-
ity to work, I st., With the aim of rational employment, retraining or
reducing the amount of work performed.
II group of disability is established for patients with III-VI stage
of the disease, requiring specic treatment, due to severe dysfunc-
tions, limitation of the ability to self-service, independent movement
of the 2nd degree, labor activity of the 2nd degree. Labor is available
only in specially created conditions.
Group I disability is established for patients with stage IV disease
in the presence of severe anemia and thrombocytopenia, persistent se-
vere infection and complications; with the rapid progression of the
disease, transformation into a malignant form, limitation of the ability
to self-service 3 tbsp., independent movement of 3 tbsp., and an unfa-
vorable prognosis.
Dispensary observation. Patients with leukemia are observed by
a local general practitioner 4 times a year (according to indications
- more often), a hematologist advises 2 times a year, an oncologist
- 3-4 times a year. Timely diagnosis of the disease, prescription of
pathogenetic therapy, exclusion of risk factors, anti-relapse treatment.
Social and labor rehabilitation includes training and retraining of pa-
tients with an accessible profession, the denition of a work recom-
mendation, rational employment, and the preparation of an IPR.
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Clinical case No. 5
1. What is the preliminary diagnosis?
Diagnosis “B-12 deciency anemia, III degree. Funicular myelo-
sis “is exposed on the basis of complaints (general weakness, poor
appetite, diculty in swallowing); the presence of syndromes of hepa-
tosplenomegaly, jaundice, polyneuropathy; physical examination data
(bright red, smooth, cracked tongue, hypotension (BP - 90/60), tachy-
cardia (Ps - 109 per minute)); laboratory tests (erythrocytes - 1.0 ×
1012 / l, hemoglobin - 40 g / l, MSM - 110 fL, color index - 1.2, ESR
- 23 mm / h, anisocytosis (macrocytosis), poikilocytosis, megalocytes,
Jolly bodies , Cabot rings). The diagnosis “B-12 induced myocardial
dystrophy, CHF II B - IIIA, FC III-IV (according to NYHA), left ven-
tricular hypertrophy, right bundle branch block” was made on the ba-
sis of complaints (shortness of breath with minimal physical exertion
(getting out of bed, dressing) , swelling of the legs, constant, slightly
increasing in the evening); examination data (the heart is enlarged by
2 cm to the left of the left midclavicular line, heart sounds are mued,
systolic non-coarse murmur over all points); data of instrumental stud-
ies (sinus rhythm, right bundle branch block, negative T wave in leads
V4-V6).
2. What additional tests would you recommend to the patient,
and what are the expected results?
In the analysis of blood - severe anemia (decrease in hemoglo-
bin to 25–40 g / l); hyper chromia - increased color index (more than
1.1); MCV over 100 ; MCH more than 35 pg per cell; the number of
leukocytes, platelets is moderately reduced; relative lymphocytosis;
hyper segmentation of neutrophils (detection of more than 5% of neu-
trophils with 5 segments or any number of neutrophils with 6 or more
segments); marked anisocytosis and poikilocytosis are noted: macro
ovalocytes are characteristic (usually found only with megaloblastic
anemia, as an exception - with myelodysplasia), schizocytes, poly-
chromatophilia, Kebot’s rings, Jolly’s bodies, normoblasts.
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The nal conrmation of the diagnosis is a sharp decrease in the
level of vitamin B12 in blood serum to 117 ± 22 pg / ml (norm 300-
900 pg / ml) and erythrocytes to 13.9 ± 3.3 pg (norm 80-300 pg). It is
possible to increase the level of indirect bilirubin and the activity of
lactate dehydrogenase (LDH) in the blood serum as a result of intra-
medullary hemolysis of erythrocytes.
If it is dicult to determine the level of vitamin B12, the tradi-
tional morphological diagnostic criterion is used - the study of bone
marrow hematopoiesis.
Instrumental studies: in the punctate of the bone marrow, a mega-
loblastic type of hematopoiesis is observed (the ratio of white and red
blood sprout is 1: 1 or 1: 2, megaloblasts are determined); trepan bi-
opsy (according to indications) is performed before the administration
of vitamin B12.
3. What is the patient’s management?
Cyanocobalamin 1000 mcg 1 time per day IM daily until hemo-
globin normalizes, maintenance therapy with Cyanocobalamin 500
mcg 1 time per month for life. Recommended: eating animal products
(liver, kidneys, eggs, milk). Elimination of alcohol intake. Treatment
of elderly people with IDA
1. Persons over 50 years of age with moderate or moderate IDA
are prescribed iron preparations at the rate of 30 mg of elemental iron
containing 400 μg (0.4 mg) of folic acid, and an additional 2.4 μg of
vitamin B12
2. Evaluation of adherence to therapy is carried out 14 days after
the start of treatment.
3. 4 weeks after the initiation of IDA treatment, Hb should be re-
determined. If there is a positive result for the prescribed treatment (an
increase in Hb by 20 g / l within 4 weeks), then the treatment should
continue for another 5 months. The general course of treatment with
iron is 6 months.
4. Within 6 months of treatment, Hb is re-determined after 3 and
6 months from the start of treatment in order to track whether hemo-
globin has returned to normal.
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380
5. In the absence of a positive result from the treatment, the doctor
should determine the possible reasons for the lack of improvement: in-
sucient dose of elemental iron, irregular intake or refusal to take iron
supplements. If errors in treatment are excluded, the patient should be
referred to a therapist or hematologist to clarify the form of anemia.
6. Persons over 50 years of age with severe anemia (Hb below 70
g / l) should be consulted by a hematologist / therapist.
7. In persons over 50 years of age with severe malnutrition, ane-
mia is often severe. However, oral iron therapy should not be started
until the patient’s appetite is restored and the patient begins to gain
weight, usually 14 days after the start of nutritional adjustments.
8. There are no special requirements for the regimen of work,
rest, treatment, rehabilitation, persons over 50 years old should refrain
from heavy physical exertion, which can potentially cause palpitations
(C).
9. The ineectiveness of IDA therapy in people over 50 is often
associated with constipation. In such cases, an adequate dose of lactu-
lose is added to the therapy at a dose of 50-100 ml, and after a stable
eect is obtained, the dose of lactulose is halved (C).
10. Diet does not play a signicant role in IDA therapy; the excep-
tion is persons over 50 years of age, adherents of vegetarianism and
other diets with a low content of iron-containing foods, who should be
advised to expand their diet by including meat products (E).
Blood transfusion is prescribed only if hemoglobin is low and
signs of an impending coma appear. For B12 deciency anemia of an
autoimmune nature, corticosteroids (prednisone) are used.
Thus, the treatment is reduced to the following principles: satura-
tion with vitamin B12, supportive measures, prevention of the devel-
opment of anemia.
Treatment of B12 deciency anemia primarily involves the de-
livery of the vitamin to the body, bypassing the gastrointestinal tract.
In case of impaired absorption of B12 by the intestines, vitamin injec-
tions are indicated, which are done daily (once or twice a day). After
ten days, the dose is reduced, the treatment continues for another ten
days. Then the drug is administered for three months once a week,
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