11.3 Chronic lymphoproliferative syndrome

CAUSES. Chronic lymphocytic leukemia, malignant lymphomas.

SYMPTOMS for chronic lymphocytic leukemia).

Leading syndromes: slow, gradual development, more often after the age of 40 years.

• anemic,

• hemorrhagic,

• immunodeficiency and inflammatory (septic conditions may develop),

• intoxication (weakness, sweating, fever),

• ossalgia, arthralgia,

• hyperplastic: a) predominant enlargement of the lymph nodes (painless, testo-elastic consistency, not soldered together, mobile);

b) the absence or moderate increase in the spleen and liver;

c) frequent skin lesions (skin infiltrates, eczema, psoriasis, herpes zoster, etc.).

• laboratory changes. KLA: leukocytosis, hyperleukocytosis mainly due to mature lymphocytes, sometimes single lymphoblasts and prolymphoblasts, Botkin-Gumprecht shadow, anemia, thrombocytopenia, suppression of other sprouts. Accelerated ESR, increased CRP and LDH. Myelogram - lymphocytes (up to 90%), lymphoblasts (up to 10%), Botkin – Gumprecht shadow (up to 10-20%). Lymph node biopsy.

pic. 7.5 Lymphadenopathy

2. Disseminated intravascular coagulation syndrome

(DIC)

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Disseminated intravascular coagulation is a complex pathological syndrome, which includes

massive blood coagulation, leading to blockage of microcirculation by loose masses of fibrin and

cell aggregates in vital organs (lungs, kidneys, liver, adrenal glands, etc.) with the development

of their dysfunction.

REASONS (etiology):

• septic (especially caused by gram-negative microorganisms);

• all types of shock (septic, cardiogenic, burn, hemorrhagic, etc.);

• malignant neoplasms (leukemia, tumors of the lungs, stomach, etc.);

• injuries (bone fractures, crash syndrome);

• obstetric pathology (premature detachment of the placenta and its presentation, embolism with amniotic fluid, severe atony of the uterus, eclampsia);

• acute intravascular hemolysis;

• immune (immunocomplex) diseases.

9. hypercoagulation; II. normal coagulation; III. hypocoagulation; IV. the outcome.

SYMPTOMS: clinical manifestations are associated with ischemic (thrombotic) and hemorrhagic injuries of organs and tissues that have a well-developed microcirculatory network (lungs, kidneys, adrenal glands, gastrointestinal tract, liver, spleen, skin, mucous membranes) and are characterized by their dysfunction and bleeding of various degrees.

One of the most common, albeit optional manifestations of this syndrome is bleeding, which is observed on average in 55-T5% of patients.

Diagnosis of ICE syndrome:

1. Vascular-platelet hemostasis. Sample pinch, tourniquet, cuff test, platelet count, platelet factor determination (TF4);

2. Coagulation hemostasis: - coagulation time;

- activated partial (partial) thromboplastin time (APTT); - determination of prothrombin time;

- determination of thrombin clotting time;

- determination of fibrinogen concentration.

3. Physiological anticoagulants:

- determination of the activity of AT III (antithrombin III).

4. Fibrinolysis;

5. Methods for detecting markers of intravascular coagulation and fibrinolysis. Paracoagulation tests:

ethanol test;

-protamine sulfate test;

- determination of RFMC (soluble fibrinmonomeric complexes); - determination of PFD (fibrin degradation products).

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7.1 TEST TASKS

(Choose one or more correct answers)

1. Manifestations of sideropenic syndrome are all signs, except

1) angular stomatitis

2) glossitis

3) dryness and hair loss

4) esophagitis

5) splenomegaly

2. Indicate the clinical manifestations of sideropenic syndrome

1) angular stomatitis

2) jaundice

3) splenomegaly

4) taste perversion

5) coilonychia

3. For the diagnosis of B12 - deficiency anemia, it is enough to identify

1) hyperchromic, hyporegenerative, macrocytic anemia

2) hyperchromic, hyporegenerative, macrocytic anemia and atrophic gastritis

3) hyperchromic, hyporegenerative, macrocytic anemia with the presence of Jolly bodies and Kebot rings in red blood cells

4. hyperchromic, hyporegenerative, macrocytic anemia and megaloblastic type of hematopoiesis

4. Petechiae are:

1. capillary expansion

2. deposition of hemosiderin in subcutaneous fat

3. extensive hematomas

4. hemorrhagic spots on the skin of a rounded shape with a diameter of 1-2 mm

5. hemorrhagic spots of irregular shape with a diameter of 3-4 mm

5. The type of bleeding characteristic of thrombocytopenia

1) hematoma;

2) petechial-spotted (microcirculatory, bruised);

3) mixed microcirculatory-hematoma (bruise-hematoma);

4) vasculitis purple;

5) angiomatous.

5. The diagnosis of leukemia is obvious in the presence of

1) anemia;

2) ulcerative necrotic lesions;

3) an increase in lymph nodes;

4) blastemia in the peripheral blood;

5) hemorrhages

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Tasks for the Hematology Section

Task 1

Patient K., 56 years old, was admitted to the clinic with complaints of: general weakness, dizziness, palpitations, shortness of breath during physical exertion, tinnitus, decreased appetite, dysphagia when eating dry, solid food (Plummer-Winson syndrome). She also began to notice hair loss and increased fragility of nails. Perversions of taste appeared (desire to eat chalk, tooth powder, raw minced meat).

The above complaints appeared during the last 6 months, in the past I didn’t get sick, menopause from 54 years, menses were plentiful for 6-7 days.

On examination: the skin and visible mucous membranes are pale, seizures in the corners of the mouth, dry, flaky skin. Spoon-shaped nails, clumsy with transverse striation (koilonychia). Smoothness of the papillae of the tongue is noted. The first tone at the apex is weakened, a gentle systolic murmur is heard there, pulse - 100 beats. in min., on the jugular vein on the right is heard the "top sound", blood pressure 110 and 60 mm Hg From other organs and systems without pathology.

Clinical blood test: Red blood cells - 2.3 x 1012 / l, Hb - 38 g / l, color index. - 0.7, white blood cells - 5.0 x 109 / l, reticulocytes - 4%. ESR - 10 mm / hour. Severe red blood cell hypochromia, anisocytosis (microcytosis), poikilocytosis.

1. What clinical and laboratory syndromes are there in the patient?

2. What research is needed to clarify the diagnosis and the causes of the disease?

Task 2

Patient S., 69 years old, was admitted to the hospital with complaints of: severe weakness, fatigue, shortness of breath when walking, palpitations, dizziness, burning tongue, decreased appetite, diarrhea, pain and numbness in the lower extremities, muscle weakness ("cotton legs" ), low-grade fever.

Sick for about 2 years, at first I did not pay attention to weakness and fatigue, then there was muscle weakness, pain and numbness in the lower extremities, in connection with which I went to the doctor and was hospitalized. Objectively: a satisfactory condition, excess nutrition. The skin is pale with an icteric shade, sclera subicteric. Heart sounds are weakened, a quiet, systolic murmur is heard at the apex, on the jugular vein on the right is a ―top sound‖, pulse -107 per minute, rhythmic, soft. The tongue is moist, bright red in color, smooth - ―lacquered‖ (due to the pronounced smoothing of the papillae), there are isolated aphthae on the mucous membrane of the cheeks. The lower edge of the liver is palpated, protruding 2.0 cm from under the edge of the costal arch. The spleen is not palpable. When striking the sternum, ribs and tibia, the patient feels soreness.

Clinical blood test: erythrocytes - 2.1 x 102 / l, Hb - 48 g / l, color index - 1.4, reticulocytes - no, platelets - 95 x 109

• l, white blood cells - 3.0 x 109 / l, ESR - 35 mm / hour. Hyperchromia of red blood cells, pronounced anisocytosis (macrocytes, megalocytes), poikilocytosis, found Jolly bodies, Kebot rings, polysegmentation of neutrophils.

1. What pathology should the patient think about?

2. What research is needed to clarify the diagnosis and etiology of the disease?

Task 3

Patient A., 19 years old, went to the doctor with complaints of: sore throat when swallowing, fever up to 39 ° C with chills, profuse night sweats, bleeding gums, nosebleeds, severe weakness, bone pain. He fell ill acutely a week ago.

Objectively: a state of moderate severity. The skin and mucous membranes are pale, the skin is wet to the touch, on the skin of the trunk, forearms and hips there are multiple subcutaneous hemorrhages. Symptoms of a ―tourniquet‖ and ―pinch‖ are positive. The submandibular, cervical, axillary and inguinal lymph nodes are palpable, pea-sized, painless, not soldered together and with skin, soft-elastic consistency, the skin above them is not changed. Bad putrefactive breath. The pharynx is hyperemic, ulcerative necrotic changes in the mucous membrane are visible on the tonsils and posterior wall of the pharynx. Gums loose, bleed easily when touched with a spatula. The painless edge of the liver is palpated, protruding 3.0 cm from the costal arch, the edge is even and soft. With percussion, the length of the spleen is 12 cm, the diameter is 8 cm. The spleen is not palpable. Rattling along the flat and tubular bones is painful.

Clinical blood test: red blood cells - 2.8 x 1012 / l, Hb - 70 g / l, color index - 0.9, platelets - 65 x 109 / l, white blood cells - 100 x 109 / l, myeloblasts - 75% , stab - 3%, segmented - 22%, eosinophils - no, basophils - no, ESR -

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56 mm / hour.

1. What syndromes can be distinguished in a patient?

2. What disease can a patient think of?

3. What research is necessary for the patient to confirm the diagnosis?

Task 4

Patient B., 61 years old, was admitted to the clinic with complaints of: general weakness, fatigue, swelling in the neck and axillary areas, increased sweating, low-grade fever.

Over the past 5 years, during preventive medical examinations, attention has been paid to an increase in the number of lymphocytes in the blood up to 40-50% with a normal number of leukocytes. The patient notes that during this period he became ill with colds more often, they last longer and harder. In the last 6 months, the above complaints appeared, in connection with which he was hospitalized.

Objectively: the condition is satisfactory. Reduced nutrition. The skin is pale. Cervical, axillary and inguinal lymph nodes the size of a bean to walnut, mobile, not soldered together, elastic-test consistency, painless, skin integument above them is not changed. The liver protrudes from under the edge of the costal arch by 2.0 cm, the edge is smooth, of a dense consistency, painless. The length of the spleen is 11 cm, the diameter is 7 cm, the edge of a dense, smooth, painless spleen is palpated.

Clinical blood test: red blood cells - 3.7 x 1012 / l, Нb - 90 g / l, color index - 0.95, white blood cells - 35 x 109 / l stab - 1%, segmented - 15%, eosinophils - 1%, lymphocytes - 80%, monocytes - 3%, ESR -35 mm / hour.

1. What syndromes can be distinguished in a patient according to a survey?

2. What research will clarify the diagnosis?

3. The presence of a symptom characteristic of this disease in a morphological study of peripheral blood? Task 5

Patient D., 52 years old, upon admission to the clinic complained of: severe general weakness, heaviness in the left hypochondrium, fever from 37.8 to 38.5 ° C with heavy sweats, and ―aches‖ in the bones.

Over the past 4 months, she had twice had a sore throat and acute respiratory illness. It was treated on an outpatient basis without significant improvement in general condition.

Objectively: a state of moderate severity. Temperature - 38.0 ° С. Reduced nutrition. The skin and visible mucous membranes are pale, on the skin of the forearms and hips there are many small bruises. Bad breath. When examining the oral cavity - the phenomenon of stomatitis and gingivitis. Axillary and inguinal lymph nodes the size of a pea, soft, mobile, painless. There is an asymmetry of the abdomen due to bulging in the left hypochondrium. The lower edge of the liver protrudes 4.0 cm from under the edge of the costal arch, smooth, painless. Percussion length of spleen - 16 cm, diameter - 10 cm, palpation of the spleen protrudes from the edge of the costal arch by 6 cm, smooth, dense, painless. Pressing and tapping on the flat and tubular bones is painful.

Clinical blood test: red blood cells - 2.5 x 1012 / l, Нb - 60 g / l, color index - 0.85, white blood cells - 200 x 109 / l, myeloblasts - 6%, promyelocytes - 4%, myelocytes - 20%, metamyelocytes - 21%, stab - 13%, segmented - 11%, eosinophils - 6.5%, basophils - 4.5%, lymphocytes - 10%, monocytes - 3%, ESR - 50 mm / hour.

1. What syndromes should be distinguished in a patient?

2. What research is needed to clarify the diagnosis?

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8. RHEUMATOLOGY AND ALLERGOLOGY