HEMATOLOGYHEMATOLOGY

Bone marrow function

Hematopoiesis Antibody producing plasma cell differentiation Monitoring hematopoietic cell quality Important key nutrients: iron

folic acid

vitamin B12

regulatory hormones

(EPO, CSF-s)

interleukins

Signs and symptoms of hematological diseases Factors

– Red blood cells– Leukocytes– Coagulation system

Alterations– Change of the amount– Change in the function

Anemia Decrease in red cell mass or hemoglobin

content of blood below the physiologic need

Not a disease itself! A clinical sign! Analysis of anaemia

– Background mechanisms loss of red blood cell - bleeding lack of red blood cell production excessive red blood cell damage

History Family history: anemia, splenomegaly, jaundice

splenectomy Bleeding tendency in the family Diet, alcohol intake Pregnancy, menorrhagia Drugs Chronic diseases Malnutrition, malabsorption Transfusion, iron or other therapy against anemia

Anemia Signs and symptoms vary with the rapidity

of onset: Rapid (bleeding or brisk hemolysis)

– cardiovascular compensatory reactions: tachycardia, postural hypotension, vasoconstriction in the skin and extremities, dyspnea on exertion, faintness, even shock

Slowly developing anemias (ie.nutritional deficiency, chr.bleeding, hemolysis etc.)– there is time for compensation– the patient remains asymptomatic for a long time

Anemia

Mild: often asymptomatic Moderate: symptoms on exertion Severe: symptoms on rest

heart failure

Anemia – non cause-specific symptoms

– fatigue– dizziness, vertigo, headache, tinnitus– increased irritability, difficult concentration– sleeping disturbancies– cold intolerance– exertional intolerance: Hb<75g/l : resting heart rate, stroke volume – dyspnea– complaints of local vascular diseases (angina pectoris, cerebral ischaemia, intermittent claudication)

Anemia - physical findingsNon cause-specific Pallor of skin and mucous membranes

– causes: Hb and blood redistribution from the skin

– colors greyish: malignancy lemon-like: hemolysis, B12 deficiency

Tachycardia Hyperkinetic precordium Systolic murmur (reversible)

Anemia - physical findingsCause-related Jaundice hemolysis Hepatosplenomegaly - e.g.hemolysis Lymphadenopathy - lymphomas,

autoimmune diseases Cheilosis (fissura) iron deficiency Koilonychia (spoon-shaped nails) iron def. Beefy red smooth tongue (Hunter-

glossitis) - pernicious anemia Neuropathy - pernicious anemia Rectal digital examination - bleeding

Polycythemias Hyperviscosity

– Decresed cerebral blood flow tinnitus, lightheadedness, dizziness, stroke– Congestive heart failure– Thrombosis

Increased cell turnover– Gout (due to hyperuricaemia)– Itching

In polycythemia vera– Thrombocytosis– Hemorrhage

Polycythemias Primary: polycythemia vera myeloproliferative disorder

– other cell lines are affected (leukocytosis, thrombocytosis)– hepatosplenomegaly– EPO level: low

Secondary: – hypoxia EPO production

chr. pulmonary diseases morbid obesitiy (Pickwick’s syndrome) high altitude

– EPO overproduction: tumors

Leukocyte disorders

Granulocytes (neutrophil, eosinophil basophil)

Monocytes and tissue macrophages Lymphocytes

Functions of leukocytes

Maintaining host defences against disease Killing microorganisms Digestion of tissue debris Releasing cytokines and mediators Activation of the immune system

Leukocyte diseases

Benign– Leukocytosis > 10 G/L– Leukocytopenia < 4 G/L– Agranulocytosis

absolute granulocyte count < 1 G/L

Malignant– Leukemias– Lymphomas

Acute leukemias

Agressive immature hemopoietic cell proliferation, without differentiation (hiatus leucemicus in the blood smear)– Subtypes:

ALL (acute lymphoblastic leukemia) ANLL (acute non lymphoblastic leukemia) or

AML (acute myeloblastic leukemia)

Acute leukemia syndrome Susceptibility to infections - serious

infections Anemia due to bone marrow infiltration

and bleeding Thrombocytopenia - bleeding tendency

purpuras, petechiae, mucosal bleeding Organ infiltration In ALL: lymphadenopathy, splenomegaly

Chronic leukemias

Uncontrolled expansion of premature hemopoietic cells which are able to differentiate

Subtypes:– Chronic myelogenous leukemia (CML or CGL)

a myeloproliferative disorder– Chronic lymphocytic leukemia (CLL)

a malignant lymphoma

Diseases of the lymphoid system Normal lymph nodes

– Non palpable or <1 cm, except in the inguinal region where can be 0,5-2 cm sized lymph nodes normally

– Small lymph nodes can be remain after infection Significant: one or more new nodes >1 cm (which

can’t explain by a previously recognised cause)– New lymph nodes in older age more significant

children more likely respond with lymphoid hyperplasia lymphyadenopathy under 30 years: 80% beningn

over 50 years: 40% beningn

Lymph node characteristics

Location Number (single, multiple, matted together)

Size Tenderness Consistency (hard, rubbery, soft)

Mobility Skin reactions above the lymph node

Malignant lymphomas

Arise in lymph nodes or extranodal lymphoid tissue

Subtypes:– Hodgkin’s disease– Non Hodgkin’s lymphomas

Hodgkin’s disease Mostly young adults Asymptomatic lymphadenopathy

– often cervical– sometimes mediastinal or abdominal

Hepatomegaly, splenomegaly Fever, night sweats, loss of weight Diagnosis: lymph node histology (Reed-Sternberg cells) Staging

Non Hodgkin’s lymphomas Older patients More diffuse lymph node involvement General symptoms:fever, weight loss, night

sweat Frequent extralymphatic involvement (50%: bone marrowanemia, tct-penia) Special forms: CLL Skin infiltration (T-cells) MALT-lymphoma Multiple myeloma

Coagulation disordersBleeding tendency, thrombosis, embolism

Factors platelets (number, function) coagulation factors (amount, function) surface of the blood vessels, circulation

Bleeding - history

History of common hemostatic stresses: gum extraction, minor surgeries menstruation, childbirth injuries

Family history of bleeding tendency

Bleeding Primary hemostatic defect (platelets)

occurs immediately after trauma from superficial sites (skin, mucous membranes, nose, rarely from

gastrointestinal, genitourinary tract)– purpuras, petechiae– ecchymoses

bleeding time

Secondary hemostatic defect (coagulation) delayed occurence (hours, days) from deep sites (joints, subcutaneous tissues, muscles,

retroperitoneum, body cavities, cerebrum)– hematomas– hemarthroses

coagulation time

Bleeding - physical findings From capillaries: purpuras, petechiae

small, superficial,dermal or mucosal pinpoint hemorrhages

characteristic for platelet disorders From small arterioles and venules:

– ecchymoses subcutaneous blood collections- bruises– hematomas deeper, palpable– in platelet and coagulation disorders

Hemarthros - bleeding into joints

characteristic for coagulation disorders (mostly hemophilia)

leads to chronic joint deformity

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Thrombosis and embolism

Virchow’s triad– Hypercoagulability– Circulation disturbance- congestion– Pathological endothelial surface