CLINICAL PATHOLOGYHematology

Drh. Ahmad Fauzi, M.Sc

Introduction

• Hematology: the study of blood and blood forming tissues.

• Blood consists of 55% plasma and 45% formed elements.

• Formed elements include erythrocytes, leukocytes, and thrombocytes.

• PLASMA – Alb, Glob, Fibrin

• BUFFYCOAT - Formed

elements include leukocytes,

and thrombocytes.

• HEMATOKRIT - Eritrosit

PLASMA

Components of Blood

• Plasma• Transport mechanism

• 90-92% water.

• 6-7% proteins (Albumin, globulin, fibrinogen)

• 2-3% • Fats

• Carbohydrates (glucose)

• Electrolytes

• Gases (O2, CO2)

• Chemical messengers

Plasma Components

Other

3%

Protein

7%

Water

90%

Serum adalah plasma darah yang telah diambil

fibrinogennya.

Fibrinogen adalah protein yang esensial dalam

dalam proses pembekuan darah.

CELLULAR

Hematology

Spleen

Kidneys

LiverBone

Marrow

Blood

HematopoieticSystem

Cellular Components

Pluripotent Stem Cell

Myeloid MultipotentStem Cells

Common LymphoidStem Cells

UnipotentProgenitors

BasophilsEosinophilsNeutrophilsMonocytes

Lymphocytes

ErythrocytesThrombocytes

Erythropoietin

WBC’sRBC’s

Platelets

ERITROSIT

Erythrocytes

• Biconcave shape.

• Diameter 7 microns.

• Cells for transport of O2 and CO2.

• Life span 120 days.

Components of Blood

• Red Blood Cells• Erythrocyte

• Hemoglobin – O2 bearing molecule• Comprised of 4

subunits:

• Globin (binds to 1 O2 molecule)

• Heme (iron)

• 100% saturation = 4 globin subunits carrying O2

• Each gram of hemoglobin = 1.34 ml O2

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Components of Blood

• Red blood cell production• Erythropoiesis

• Erythropoietin

• Hemolysis

• Sequestration

• Laboratory analysis of red blood cells• Red blood cell count

• Hematocrit

• Hemoglobin

Diseases of Erythrocytes

• Anemia ( RBC, Hb & Hematokrit)

• Anemias• Anemia is a sign, not a separate disease process.

• Signs and symptoms may not be present until the body is stressed.

• Differentiate chronic anemia from acute episode.

• Treat signs and symptoms.• Maximize oxygenation and limit blood loss.

• Establish IV therapy if indicated.

ABNORMALITAS MORFOLOGI

ABNORMALITAS MORFOLOGI

Diseases of Erythrocytes

Brady; Paramedic Care Principles and Practice

Diseases of Erythrocytes

• Sickle Cell Disease• Normal red cells maintain

their shape as they pass through the capillaries and release oxygen to the peripheral tissues (upper panel). Hemoglobin polymers form in the sickle rell cells with oxygen release, causing them to deform. The deformed cells block the flow of cells and interrupt the delivery of oxygen to the tissues (lower panel).

Diseases of Erythrocytes

• Sickle Cell Disease (cont.)• Sickle cell crises

• Vaso-occlusive

• Musculoskeletal/abdominal pain

• Priapism

• Renal/cerebral infarctions

• Hematological• Lowered hemoglobin

• Splenic sequestration

• Infectious

• Management• Follow general treatment guidelines prn.

• Consider analgesics.

Diseases of Erythrocytes

• Polycythemia• Overproduction of erythrocytes.

• secondary dehydration.

• Most deaths due to thrombosis

• Results in bleeding abnormalities:• Epistaxis, spontaneous bruising, GI bleeding.

• Management:• Follow general treatment guidelines.

Hematokrit (HCT)/ Packed cell volume (PCV)

• Hematokrit Adalah persentase sel darah merah tehadap volume darah total.

• Penurunan nilai Hct merupakan indikator anemia (karena berbagai sebab), reaksi hemolitik, leukemia, sirosis, kehilangan banyak darah dan hipertiroid.

• Peningkatan nilai Hct dapat terjadi pada eritrositosis, dehidrasi, kerusakan paru-paru kronik, polisitemia dan syok.

Know Normal Ranges!!!

WBC

RBC

HGB

HCT

MCV

MCH

MCHC

PLT

MPV

SEGS

LYMPHS

MONOCYTES

EOSINOPHILS

BASOPHILS

Mean Corpuscular Volume (MCV) (Volume korpuskuler rata – rata)• MCV adalah indeks untuk menentukan ukuran sel darah merah.

• MCV menunjukkan ukuran sel darah merah tunggal apakah sebagai

Normositik (ukuran normal)

Mikrositik (ukuran kecil < 80 fL), atau

Makrositik (ukuran kecil >100 fL).

• Penurunan nilai MCV terlihat pada pasien anemia kekurangan besi, anemia pernisiosa dan talasemia, disebut juga anemia mikrositik.

• Peningkatan nilai MCV terlihat pada penyakit hati, alcoholism, terapi antimetabolik, kekurangan folat/vitamin B12, dan terapi valproat, disebut juga anemia makrositik

Mean Corpuscular Hemoglobin Concentration (MCHC) (Konsentrasi Hemoglobin Korpuskuler rata – rata)

• Indeks MCHC mengukur konsentrasi Hb rata-rata dalam sel darah merah; semakin kecil sel, semakin tinggi konsentrasinya.

• Perhitungan MCHC tergantung pada Hb dan Hct.

• Penurunan MCHC (hipokromik) terjadi pada pasien kekurangan besi, anemia mikrositik, anemia karena piridoksin, talasemia

• Peningkatan MCHC (hiperkromik) terjadi pada sferositosis, bukan anemia pernisiosa.

Classifications of Anemias

Microcytic, Hypochromic• Iron deficiency

• Sideroblastic

• Chronic disease, Inflammation

• Lead poisoning

• Thalassemia trait

Microcytic, Hypochromic

• Many RBCs smaller than

nucleus of normal

lymphocytes, increased central pallor.

• Iron deficiency, thalassemias, anemia of chronic disease.

Classifications of Anemias

Normochromic• Hereditary Spherocytosis

• Hereditary Elliptocytosis

• PNH

• G6PD deficiency

• Aplastic anemia

• Acute blood loss

Classifications of Anemias

Macrocytic• Vitamin B12 deficiency

• Folate deficiency

• Liver disease

Macrocytic RBCs

• Most RBCs larger than nucleus of normal

lymphocytes, increased MCV.

• Folate or Vitamin B12 deficiencies, alcoholism, and liver disease.

Reticulocytes

• Immature RBCs.

• Contain residual

ribosomal RNA.

• Reticulum stains blue using a supravital stain (new methylene blue).

• Counted and expressed as % of total red cells.

LEUKOSIT

Components of Blood

• Leukocytes (cont.)• White Blood Cell Count

• Normal 5-9 k WBC’s

• Leukopoiesis• Granulocytes

• Neutrophil

• Basophil

• Eosinophil

• Monocytes

• Lymphocytes

MyelocyticMaturation Series

Myeloblast

Promyelocyte

Myelocyte

Metamyelocyte

Band Neutrophil

Segmented Neutrophil

Leukocytes

• Five types.

• Size 8-20 microns.

• Involved in fighting infection, combatting allergic reactions, and immune responses.

Components of Blood

• White Blood Cells (Leukocytes)• Margination

• Phagocytosis

The macrophage is using its internal

cytoskeleton to envelop cells of the fungus

Candida albicans.

Components of Blood

• Leukocytes (cont.)• Immunity

• Subpopulation of lymphocytes known as T cells and B cells

• T cells develop cellular immunity.

• B cells produce humoral immunity

Components of Blood

• Leukocytes (cont.)• Autoimmune disease

• May be specific or general

• Alterations in the immune process• Immunosuppression

• HIV

• Anti-rejection medication

• Chemotherapy/Cancer

Components of Blood

• Inflammatory Process• MAST CELLS.

• Immunoglobulin E antibody IgE,

• Antigens• Antigens stick to the mast cell IgE

antibodies, causing granules in the mast cell to fire their contents into the surrounding tissue.

• This releases a host of inflammatory materials - leukotrienes, tumor necrosis factor, interleukin-4 and other cytokines that turn on other inflammatory cells.

• These materials cause fluid to leak from the capillaries and white cells including neutrophils, T cells and eosinophils to leave the circulation. The end result is a "local inflammatory response", a red, itchy welt.

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Diseases of Leukocytes

• Leukopenia/Neutropenia• Too few white blood cells or neutrophils.

• Follow general treatment guidelines and provide supportive care.

• Leukocytosis• An increase in the number of circulating white blood

cells, often due to infection.• Leukemoid reaction

Diseases of Leukocytes

• Leukemia• Cancer of hematopoietic cells

• Initial presentation• Acutely ill, fatigued, febrile and weak, anemic.

• Thrombocytopenia

• Often have a secondary infection.

• Management• Follow general treatment guidelines.

• Utilize isolation techniques to limit risk of infection.

Diseases of Leukocytes

• Lymphomas• Cancers of the lymphatic system

• Hodgkin's

• Non-Hodgkins

• Presentation• Swelling of the lymph nodes

• Fever, night sweats, anorexia, weight loss, fatigue, and pruritis

• Management• Follow general treatment guidelines.

• Utilize isolation techniques to limit risk of infection.

Toxic Granulation

• Increased basophilic granules

in neutrophils.

• Seen in severe infections, burns, malignancies, and pregnancy.

• Distinguish from basophils.

Dohle Bodies

• Sky blue inclusions in cytoplasm of neutrophils.

• Seen in infections, burns, myleproliferative disorders, and pregnancy.

• Composed of RER and glycogen granules.

TROMBOSIT

Thrombocytes

• Smallest cells in the blood.

• Active role in coagulation and hemostasis.

Components of Blood

• Platelets (Thrombocytes)• Megakaryocytes

• Thrombopoietin

• Thrombocytopenia

• Thrombocytosis

Components of Blood

• Hemostasis- 3 mechanisms• Vascular spasm

• Contraction of tunica media

• Platelet plug• Platelet aggregation

• Coagulation• Formation of fibrin clot

This scanning electron micrograph shows the fine structure of

a blood clot. Platelets released from the circulation and exposed

to the air use fibrinogen from the blood plasma to spin a mesh

of fibrin.

Components of Blood

• Hemostasis (cont.)• Fibrinolysis

• Lysis of clot (plasmin)

• Thrombosis• Thrombolytics

• Medications affecting clot formation• Alter the enzyme

on the platelet.

• Affect the coagulation cascade.

• Enhance clotting.

Coagulation Cascade - Synopsis

Clotting Disorders

• Thrombocytosis and Thrombocytopenia• Thrombocytosis

• An abnormal increase in the number of platelets

• Thrombocytopenia• An abnormal decrease in the number of platelets

• Sequestration

• Destruction (ITP)

• Decreased production

• Management• Provide supportive care and follow general treatment

guidelines.

Clotting Disorders

• Hemophilia• Deficiency or absence of a blood clotting factor

• Deficiency of factor VIII causes hemophilia A.

• Deficiency of factor IX causes hemophilia B.

• Deficiency is a sex-linked, inherited disorder.• Defective gene is carried on the X chromosome.

• Signs & Symptoms• Numerous bruises, deep muscle bleeding, and joint bleeding.

Clotting Disorders

• Hemophilia (cont.)• Management

• Treat the patient similarly to others.• Administer supplemental oxygen.

• Establish IV access.

• Be alert for recurrent or prolonged bleeding, and prevent additional trauma.

• Von Willebrand’s Disease• Deficient component of factor VIII

• Generally results in excessive bleeding.

• Generally is not serious; provide supportive care.

Clotting Disorders

• Disseminated Intravascular Coagulation• System activation of coagulation cascade.

• Results from sepsis, hypotension, OB complications, severe tissue or brain injury, cancer, and major hemolytic reactions.

• Multiple Myeloma• Cancerous disorder of plasma cells.

• Pathologic fractures are common.

TERIMA KASIH

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