kul kel hematologi sem v.ppt

8/11/2019 Kul Kel hematologi Sem V.ppt

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Indriani SilviaPatologi Klinik

FK UNSWAGATI18 Oktober 2013

BLOK 254



Kelainan pada hematologiJenis kelainan hematologi

Gambaran kelainan hematologi padapemeriksaan laboratorium

Kelainan onkologiJenis pemeriksaan onkologi

Indikasi dan tujuan pemeriksaanInterpretasi hasil pemeriksaan



Transports oxygen, called oxyhemoglobin,when it gives up its oxygen it isdeoxyhemoglobin.

Also binds and transports carbon dioxide,carbaminohemoglobin.

Makes up ± 97 % of RBC ± 250 million Hbmolecules per RBC

Men: 14-18 mg/dl blood

Women: 12-16 mg/dl blood



Types of Hemoglobin (Hb)

Hb A

• 96% of adult Hb (α2 β2)

Hb A2• 3% of adult Hb (α2 σ2)

Hb F

• 1 % of adult Hb (α2 γ2)



• Liver – contains phagocytic cells known as

Kupffer cells that act as a filter for damaged or

aged cells in a manner similar to, but less

efficient than the phagocytic cells in thespleen. If the bone marrow cannot keep up with the physiologic

demand for blood cells, the liver may resume the

production of blood cells that it began during fetal life



Bloodgroup

Antigen Antibody Donor to Recipientfrom

A A Anti-B A A, O

B B Anti-A B B, O

AB AB Neither AB A, B, AB, O

O Neither Anti-A/Anti-B

O, A, B, AB O

Universal donor "O"Universal recipient "AB"



Acute Hemolytic Reaction Symptoms

• Fever, chills and fever, the feeling of heat along thevein in which the blood is being transfused

• Pain in the lumbar region• Constricting pain in the chest, tachycardia, hypo-

tension• Hemoglobinemia with subsequent hemoglo-binuria

and hyperbilirubin-emia.

"feeling of impending doom"



Acute Hemolytic Reaction

Causes

• Human error!

Transfused red cells react with circulating antibody in therecipient with resultant intravascular hemolysis



Acute Hemolytic Reaction

Frequency

• Rare

Prevention

• Proper identification of patients, pretransfusion blood

samples and blood components at the time of

transfusion



Delayed Hemolytic ReactionFalling hematocrit

due to extravascular destruction of the transfused redblood cells)

Positive direct antiglobulin (Coombs) test (DAT)

Occurs about 4-8 days after blood transfusion

Patients may manifest fever and leukocytosis

Appearing to have an occult infection.



Febrile Transfusion Reaction

Fever or chill fever

• temperature rise of 1.5 F or 1.0 C from the baseline

Cytokines and antibodies to leukocyte antigensreacting with leukocytes or leukocyte fragments

1 in 8 transfusions



Allergic – urticaria

Laryngeal edema and bronchospasm

1% of recipients

• If coupled with another sign, such as fever, evaluationfor a hemolytic reaction may be indicated.



Allergic – Anaphylaxis

Anaphylactic or anaphylactoid

• Respiratory involvement with dyspnea or stridor

Cardiovascular instability

• hypotension, tachycardia, loss of consciousness,

cardiac arrhythmia, shock and cardiac arrest



Volume Overload Transfusion-related volume overload

Infuse smaller volumes more slowly



Bacterial Contamination

Hypotension, shock, fever and chills, nausea and

vomiting, and respiratory distress

Gram stain and blood culture



Follow protocol for transfusion reactions

implemented by the institution



Stop the transfusion immediately!

Disconnect the intravenous line from theneedle.

Seek medical attention immediately. If thepatient is suffering cardiopulmonary collapse,and medical attention is not immediately

available, press for ―Code"



Check to ensure that the patient name and

registration number on the blood bag label

exactly with information on the patient's

identification

Do not discard the unit of blood that has been

discontinued because it may be necessary for

the investigation of the transfusion reaction.



Reaction Type Treatment - Adult Pediatric Follow-up

Acute

Hemolytic

Reactions

Diuretic therapy: Initially, give 40-80

mg Furosemide (Lasix)

intravenously. This dose can be

repeated once. Lack of response

to furosemide in 2-3 hours

indicates the presence of acute

renal failure.

Pediatric dose: 1-2

mg/kg/dose.

May repeat once

at 2-4 mg/kg.

Treat shock and disseminated

intravascular coagulation with

appropriate measures if and when

they appear.

Water loading: The patient should be

hydrated to maintain urinary

output of at least 100 mL/hr until

urine is free of hemoglobin.

Infuse a loading dose of 0.9% sodium

chloride or 5% dextrose in 0.45%

sodium chloride. Chart hourly

urine output. Maintain the urine

output by administeringintravenous fluid at 100 mL/hour

until the urine is free of

hemoglobin. If the patient's

urinary output does not increase,

with this hydration any additional

fluids should be infused with

caution.

Pediatric patients

should receive a

smaller loading

volume of fluid

in proportion to

their body

surface area.



Delayed

Hemolytic

Transfusion

Reactions

Specific treatment generally is not

necessary

Supplemental transfusion of blood lacking

the antigen corresponding to the

offending antibody may be necessary

to compensate for the transfused

cells that have been removed fromthe circulation.





Allergic

Transfusion

Reactions

Antihistamines(e.g., Benadryl). Give

50-100 mg orally or intravenously. If

urticaria develops slowly,

antihistamines may be given orally.

Pediatric dose: 1-2

mg/kg

intramuscularly or

intravenously for 25-

50 mg per average

dose.

Routine use of Benadryl as premedication

for all transfusions, regardless of a history

of allergic reactions, is discouraged.

Aminophylline for wheezing, at a dose

of 125-250 mg intravenously slowly

over a period of about five minutes

Pediatric dose: 3

mg/kg/dose in

intravenous drip over

of 20 minutes.

Epinephrine for severe, acute reactions

including laryngeal edema or

bronchospasm Give 0.1-0.5 mg (0.1-0.5

mL of a 1:1000 solution)

subcutaneously. Subcutaneous dose

may be repeated at 10-15 minute

intervals. The total subcutaneous dose

in a 24-hour period, with rare

exception, should not exceed 5 mg.

Pediatric dose: 0.03

mL/M2 (0.03 mg/M2

of a 1:1000 solution)

given subcutaneously.

A single pediatric

dose should not

exceed 0.3 mg.



Febrile

Transfusion

Reactions

Premedicate the patient with

acetaminophen or other

antipyretic agents when previous

reactions have been extremely

bothersome. Pediatric dose: 10

mg/kg to a maximum of 600 mg.

Aspirin will adversely affect the patient's

platelet function, so non-aspirin

antipyretic agents are preferable.

Severe shaking

Chills

(rigors) can be controlled by the

sedative effect of Benadryl or

Demerol (25-50 mg given

intramuscularly or intravenously

Note: Demerol may cause acute

respiratory arrest. An opiate

antagonist (Narcan) should be

immediately available.

Sepsis Due to

Bacterial

Contaminationof Donor Blood

Treatment of septic shock includes:

terminating the suspected

transfusion immediately, cardio-vascular and respiratory support,

blood culture of the patient, and

administration of broad spectrum

antibiotics including anti-

pseudomonas coverage if the

blood component involved is Red

Blood Cells.




Abnormally low number of RBC or Hb levels

Reduced oxygen carrying capacity

Causes

Blood loss Increased rate of red cell destruction

• Hemolytic anemia

Deficient or impaired red cell production



Risk factors Poor diet Intestinal disorders Menstruation Pregnancy Chronic conditions Family history



―NOT A DISEASE‖ but a symptom

• Dependent on severity, speed of development, age,

health status and compensatory mechanisms

• Associated with impaired O2 transport, alteration inRBC structure or with chronic illness

• Not expressed until 50% of RBC mass is lost



Signs and symptoms The main symptom of most types of anemia is

fatigue• Weakness

• Pale skin• Tachycardia• Shortness of breath• Chest pain• Dizziness

• Cognitive problems• Numbness or coldness in your extremities• Headache



Iron Deficiency Anemia Most common form of anemia

• Affects about one in five women• Half of pregnant women and 3 percent of men in the

United States. The cause is a shortage of the element iron

• Nutritional imbalance• Slow, chronic bleeding disorders• Inability to recycle plasma iron



Vitamin Deficiency Anemias Folate and vitamin B-12 deficiency Intestinal disorder that affects the absorption of

nutrients Fall into a group of anemias called megaloblastic

anemias, in which the bone marrow produceslarge, abnormal red blood cells.



Anemia of Chronic Disease Interfere with the production of red blood cells,

resulting in chronic anemia Kidney failure also can be a cause of anemia

• The kidneys produce a hormone called erythropoietin,which stimulates your bone marrow to produce redblood cells. A shortage of erythropoietin, which can result from kidney failure

or be a side effect of chemotherapy, can result in a shortage ofred blood cells.



Aplastic Anemia Life-threatening anemia caused by a decrease in

the bone marrow's ability to produce all threetypes of blood cells — red blood cells, whiteblood cells and platelets

Cause of aplastic anemia is unknown• autoimmune disease

• Chemotherapy

• Radiation therapy• Environmental toxins



Anemias associated with bone marrow diseaseLeukemia and myelodysplasia, can cause

anemia by affecting blood production in the bonemarrow

Effects vary from a mild alteration in blood

production to a complete, life-threateningshutdown of the blood-making process Myelodysplasia is a pre-leukemic condition

that can cause anemia.Other cancers of the blood or bone marrow, such as

multiple myeloma, myeloproliferative disorders orlymphoma, can cause anemia.



Hemolytic Anemias Red blood cells are destroyed faster than bone

marrow can replace them. Autoimmune disorders can produce antibodies to

red blood cells, destroying them prematurely• Hemolytic anemias may cause yellowing of the skin

(jaundice) and an enlarged spleen.



Hereditary Spherocytosis

Mutations in the ankyrin molecule with a

secondary deficiency of spectrin along the cell

membrane• Reduced red cell stability

Does not affect oxygen carrying capacity

Splenic sequestration



Sickle cell anemia Defective form of hemoglobin that forces red

blood cells to assume an abnormal crescent(sickle) shape.

• Mutation for the gene coding for the β-globulin chain Valine is substituted for glutamic acid HbS

Red cells die prematurely, resulting in a chronicshortage of red blood cells.

• Block blood flow through small blood vessels in thebody, producing other, often painful, symptoms.



Single base pair mutation results in a

single amino acid change.

Under low oxygen, Hgb becomes insoluble

forming long polymersThis leads to membrane changes

(―sickling‖) and vasoocclusion



Deoxygenation of SS erythrocytes leads tointracellular hemoglobin polymerization, loss ofdeformability and changes in cell morphology.

OXY-STATE DEOXY-STATE



α- Thalassemia

Common in Asians

Deletion of glubulin chain loci

4 possible degrees of α thalassemia:• Silent carrier, loss of a single α globulin gene

• α thalassemia trait, loss of a pair of globulin gene

• HbH disease, only a single gene is present

• Hydrops fetalis, deletion of all α globulin



The only treatments are stem cell

transplant and simple transfusion.

Chelation therapy to avoid iron overload

has to be started early.

TALASSEMIA



An acquired disorder of the bone marrow that

causes the overproduction of all three blood cell

lines

• white blood cells, red blood cells, and platelets It is a rare disease that occurs more frequently in

men than women, and rarely in patients under 40

years old.

causes is unknown



Usually develops slowly, and most patients are

asymtomatic

• abnormal bone marrow cells proliferate uncontrollably

leading to acute myelogenous leukemia Patients have an increased tendency to form

blood clots that can result in strokes or heart

attacks

• Some patients may experience abnormal bleedingbecause their platelets are abnormal



Symptoms Headache Dizziness Pruritus Fullness in the left upper abdomen Erythema (face) Shortness of breath Orthopnea Symptoms of phlebitis



Collectively known as White Blood Cells (WBC)

Formed elements of the blood with organellesand a nucleus but lack hemoglobin

Protect the body against microorganisms andremove dead cells and debris from the body



Per µl blood Per µl of blood

Total WBC count 5,000 – 10,000

Neutrophils 50 - 70% 2,000 – 7,000

Lymphocytes 20 - 40% 1,000–

4,000Monocytes 1 – 6% 50 – 600

Eosinophils 1 – 5% 50 – 500

Basophils 0 – 2% 0 - 100



Leukopenia

• Decreased peripheral white cell count due to decrease

numbers of any specific types of leukocytes

Leukocytosis• Non –neoplastic elevation of WBC count



Neutropenia

Reduction in the number of granulocytes

(<1500/µl)

Increased risk of infection• Reduced phagocytosis response



Neutropenia

Decreased or defective granulopoiesis

• Aplastic anemia

• Anti-neoplastic agents• Other drugs: chloramphenicol, sulfonamides,

chlorpromazine

Accelerated removal or destruction

• Aggressive and chronic infections



Manifestation of Neutropenia

Infections

Signs and Symptoms Malaise, chills, fever

Ulcerative necrotizing lesions of the mouth, skin

vagina and GI tract



Reactive Leukocytosis

Increase number of WBC

Common reaction due to a variety of

inflammatory states caused by microbial or non-microbial stimuli

Usually non-specific



Causes of Leukocytosis

Polymorphonuclear leukocytosis Acute bacterial infections

Eosinophilic leukocytosis Allergic disorders

Monocytosis Chronic infections

Lymphocytosis Chronic immunologic disease



Leukemoid reaction – this is an extreme neutrophilia with a WBCcount > 30 x 109/L

Many bands, metamyelocytes, and myelocytes are seen

Occasional promyelocytes and myeloblasts may be seen.

This condition resembles a chronic myelocytic leukemia (CML), butcan be differentiated from CML based on the fact that in leukemoidreactions:

There is no Philadelphia chromosome

The condition is transient

There is an increased leukocyte alkaline phosphatase score (moreon this later)

Leukemoid reactions may be seen in tuberculosis, chronicinfections, malignant tumors, etc.



1. Leukemia – neoiplasms of the hematopoietic

stem cells

2. Malignant lymphomas – cohesive tumor

lesions; neoplastic lymphocytes3. Plasma cell dyscrasias – arising from the

bones; localized disseminated proliferation of

antibody forming cells

4. Histocytoses – proliferative lesions ofhistiocytes



Leukemia

Malignant neoplasm of the hematopietic stem

cells

BM replaced by unregulated, proliferating,immature neoplastic cells blood leukemia

enter spleen, lymph nodes

Most common cancer in the paediatric age

Leading cause of death in children between 3and 14 years old



Classification of Leukemia A. According to cell type and state of cell maturity

• Lymphocytic – immature lymphocytes and theirprogenators

• Myelocytic – pluripotent myeloid stem cells andinterferes with maturation of all granulocytes, RBCand platelets

B. Acute or Chronic

• Acute – immature cells (blast)• Chronic – well differentiated leukocytes



Congenital• Pelger-Huet anomaly

Bilobed and occasional unsegmented neutrophils

Autosomal recessive disorder



• Neutrophil hyper-segmentation Rare autosomal dominant condition

Neutrophil function is essentially normal

• May-Hegglin anomaly

Neutrophils contain basophilic inclusions of RNA Occasionally there is associated leucopenia

Thrombocytopenia and giant platelet are frequent



• Alder’s anomaly Granulocytes, monocytes and lymphocytes contain granules which stain

purple with Romanowsky stain

Granules contain mucopolysaccharides



• Chediak-Higashi syndrome Autosomal recessive disorder

Giant granules in granulocytes, monocytes and lymphocytes

Partial occulocutaneous albinism

Depressed migration and degranulation Recurrent pyogenic infections

Lymphoproliferative syndrome may develop

Treatment is BMT



Acquired Toxic granulation

Dohle bodies

Pelger cells

Hypersegmented neutrophils



Leucocyte adhesion deficiencyChronic granulomatous disease

Chediak-Higashi syndrome

Primary immunodeficiency Severe combined immunodeficiency

Common variable immunodeficiency

Isolated IgA deficiency

T-cell immunodeficiency

Thymic aplasia (Di George syndrome)



Block in the differentiation of leukemic cells with

prolonged genration time clonal expansion of

the transformed stem cells + failure of maturation

accumulation of leukemic blast

suppressnormal hematopoietic stem cells



Features

Sudden onset (3 months)

Depressed marrow function

Bone pain and tenderness Generalized lymphadenophaty

Splenomegaly, hepatomegaly

CNS: headache, vomiting



Most common leukemia in children (80%)

Treatable and potentially curable

Classified according to lymphocytes and state

of maturation1. Early B cell

2. Pre-B cell

3. Mature B cell

4. Early T cell5. Mature T cell



Acute Non-lymphocytic Leukemia (ANLL)

Most common in adults; >50% 60years old

70% of adults will enter remission with induction

chemo• 25-35% of those in remission will have a 5 year

survival rate

BM transplant



Treatment

Selective radiation

Chemotherapy

1. Induction2. Intensification

3. Maintenance and consolidation

Bone marrow transplant



Insidious onset

Incidental findings during routine exam



Proliferation and accumulation of mature

lymphocytes which are immunologically

incompetent

• B cell line (US)• T cell line (Asia)

Hairy cell leukemia



15% of all leukemias

Chromosomal abnormality (Ph1)

Mostly B cell disease

• Leukocytosis• Splenomegaly

• Hepatomegaly

• Lympadenopathy

Bone marrow transplant 5 year survival for 50-75% of patients



Two distinct phases

Chronic

• Last about 3-4 years

•Near end

accelerated phase: fever, night sweats,malaise

Acute

• 2-4 months

• Poor prognosis, palliative management



Primary solid tumors of the lymphoid system

Cancers involving lymphocytes during

maturation or storage in the bone marrow

Third most common malignacy in children



Hodgkin’s Lymphoma

Disorders primarily involving the lymphoid

tissues

Anatomical spread Morphological presence of Reed-Sternberg cells

60-90% cure rate



Manifestations of Hodgkin’s

A symptoms

• Painless progressive enlargement of a single or group

of nodes (neck)

• May spread continuously through out the lymphatic

system

B symptoms

• Fever, night sweat, weight loss

• Fatigue, anemia



Treatment for Hodgkin’s

Radiation

Chemotherapy



Non-Hodgkin’s Lymphoma

Involves lymphoid tissue and may spread to

various tissues

Mostly B cell (80%) Cause may be viral or genetic

• EBV

• Immunosuppresed patients

AIDS After organ transplant



Treatment

Early stage radiation

Late stage chemo and radiation

BM transplant



Transfuse for any severe anemia with

physiologic compromise.

Decide early whether transfusion will be

rare or part of therapy. Avoid long-term complications by working

with your blood bank and using chelation

theraoy.

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