Patho Ii Blood Alterations

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CYTIC AND CHROMIC

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size of cell and hemoglobin content

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MACROCYTIC NORMOCHROMIC ANEMIA

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Pernicious anemia- lack of vit B 12 (cobalamin)
Folate deficiency anemia- lack of folate

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PERNICIOUS ANEMIA

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lack of vitamin B12 (cobalamin) for erythropoiesis, abnormal DNA and RNA synthesis in the erythroblast, premature cell death
PRIMARY CAUSE- congenital or acquired deficiency of intrinsic factor (IF); genetic disorder of DNA synthesis

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FOLATE DEFICIENCY ANEMIA

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-lack of folate for erythropoiesis; premature cell death
PRIMARY CAUSE- dietary folate deficiency

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MICROCYTIC HYPOCHROMIC ANEMIA

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small abnormally shaped erythrocytes and reduced hemoglobin <>
Examples- iron deficiency, Sideroblastic anemia, Thalassemia

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IRON DEFICIENCY ANEMIA

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lack of iron for Hgb production, insufficient Hgb
PRIMARY CAUSES- chronic blood loss, dietary iron deficiency disruption of iron metabolism or iron cycle

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SIDEROBLASTIC ANEMIA

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-dysfunctional iron uptake by erythroblasts and defective porphyrin and heme synthesis
PRIMARY CAUSES- congenital dysfunction of iron metabolism in erythroblasts, acquired dysfunction of iron metabolism as a result of drugs or toxins

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THALASSEMIA

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impaired synthesis of alpha or beta chain of hemoglobin A; phagocytosis of abnormal erythroblasts in the marrow
PRIMARY CAUSE- congenital genetic defect of globin synthesis

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NORMOCYTIC NORMOCHROMIC ANEMIA

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normal size, normal Hgb <>
Examples- Aplastic anemia, posthemorrhagic anemia, hemolytic anemia, sickle cell anemia, anemia of chronic disease

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APLASTIC ANEMIA

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insufficient erythropoiesis
PRIMARY CAUSE- depressed stem cell proliferation resulting in bone marrow aplasia

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POSTHEMORRHAGIC ANEMIA

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blood loss
PRIMARY CAUSE- acute or chronic hemorrhage that stimulates increased erythro poiesis, which eventually depletes body iron

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HEMOLYTIC ANEMIA-

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normocytic normochromic
-premature destruction (lysis) of mature erythrocytes in the circulation
PRIMARY CAUSE- increased fragility of erythrocytes

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SICKLE CELL ANEMIA

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normocytic normochromic
-abnormal cell shape with susceptibility to damage, lysis, and phagocytosis
PRIMARY CAUSE- congenital dysfunction of hemoglobin synthesis

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ANEMIA OF CHRONIC DISEASE

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normocytic normochromic
-abnormally increased demand for new erythrocytes
PRIMARY CAUSE- chronic infection or inflammation; malignancy

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DECREASED OR DEFECTIVE PRODUCTION OF ERYTHROCYTES

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Altered Hgb synthesis (iron deficiency, thalassemia, anemia of chronic inflammation)
Altered DNA synthesis resulting from deficient nutrients.
Pernicious anemia
Stem cell dysfunction (aplastic anemia, myeloproliferative leukemia)
Bone marrow infiltration (carcinoma, lymphoma)
Pure red cell aplasia

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INCREASED ERYTHROCYTE DESTRUCTION

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Blood loss (acute hemorrhage, trauma)
Hemolysis (intracorpuscular defect) - membrane- hereditary spherocytosis - Hemoglobin- sickle cell trait or disease - Glycolysis- pyruvate kinase - Oxidation- G6PD deficiency
Hemolysis extracorpuscular defect (immune mechanisms - warm antibody/cold antibody, Infection- clostridial, malarial, - trauma to erythrocyte- hemolytic uremic syndrome
Splenic sequestration- hypersplenism

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ANISOCYTOSIS

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assuming various sizes

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POIKILOCYTOSIS

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assuming various shapes

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INCREASE IN 2,3 DPG

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increase in release of oxygen from hgb

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PERNICIOUS ANEMIA

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CLINICALLY, THE DIS. DEVELOPS SHOWING VAGUE SYMP.
(INFECTIONS, MOOD SWINGS AND RENAL, CV OR GI PROBLEMS)
TILL Hb. IS  TO 7-8 g/dl WHEN THE TYPICAL SYMPTOMS APPEAR:
WEAKNESS, FATIGUE, DISTAL PARESTHESIAS AND THE NEUROLOGICAL MANIFESTATIONS DUE TO THE DEMYELINIZATION OF THE LATERAL AND POST. COLUMNS OF THE SPINAL CORD
(ATAXIA, SPASTICITY AND
LOSS OF THE VIBRATION AND POSITION SENSE).
ALSO ANOREXIA, ABDOMINAL PAIN,
WEIGHT LOSS AND A SORE TONGUE.

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DIAGNOSIS OF PERNICIOUS ANEMIA

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ESTABLISHED BY THE CLINICAL MANIFESTATIONS,
BLOOD TESTS, BONE MARROW ASPIRATION AND THE SCHILLING TEST WHICH MEASURES THE URINE EXCRETION OF PREVIOUSLY ADM. RADIOACTIVE COBALAMIN
INDICATION OF ABSORPTION.
LOW URINARY EXCRETION
LOW ABSORPTION
 PERNICIOUS ANEMIA.
A SECOND TEST IS GIVEN WITH ADM. OF IF. AN  IN URINARY EXCRETION CONFIRMS THE DIAG.

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FOLATE DEF. ANEMIAS

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FOLIC ACID IS ESSENTIAL TO ERYTHROPOIESIS
(IT IS A COMPONENT OF THYMINE, ADENINE AND GUANINE,
INDISPENSABLE IN DNA SYNTH.).
HUMANS NEED TO GET FROM THE DIET WITH DAILY REQ. OF 50-200 g.
FOLATE DEF. IS  IN ALCOHOLIC PERSONS, CHRONICALLY MALNOURISHED INDIVIDUALS AND THOSE WHO MAINTAIN
DIETS LOW IN VEGETABLES

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CLINICAL MANIFESTATIONS AND TREATMENT OF FOLATE DEFICIENCY

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CLINICAL MANIFESTATIONS ARE SIMILAR TO THOSE OF PERNICIOUS ANEMIA
PLUS SEVERE STOMATITIS
(PAINFUL ULCERATIONS OF THE BUCCAL MUCOSE AND TONGUE) DYSPHAGIA, FLATULENCE AND LIQUID DIARRHEA.
NEUROLOGIC MANIFESTATIONS ARE NOT TYPICALLY PRESENT.

TREATMENT REQUIRES DAILY ADM. OF ORAL FOLATE PREPARATIONS
(1 mg NORMALLY, 5 mg FOR ALCOHOLICS)
AND CLINICAL MANIFESTATIONS USUALLY DISAPPEAR IN 1-2 WEEKS.

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MICROCYTIC-HYPOCHROMIC ANEMIAS

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DISORDERS OF Fe2+ METABOLISM
--DISORDERS OF PORPHYRIN AND HEME SYNTH.
--DISORDERS OF GLOBIN SYNTH

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IRON DEF. ANEMIA

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IT’S THE MOST COMMON TYPE WORLDWIDE. IN DEVELOPING COUNTRIES USUALLY RESULTS FROM PARASITIC INFESTATIONS (HOOKWORM) AND  INGESTION OF Fe DUE TO MALNUTRITION.
IN WELL-DEVELOPED SOCIETIES THE MOST COMMON CAUSES ARE PREGNANCY AND CHRONIC BLOOD LOSS
(GASTRIC OR DUODENAL ULCERS AND CANCER, ULCERATIVE COLITIS AND MENORRHAGIA). Young women always at risk.
A BLOOD LOSS OF 2-4ml/day (1-2 mg OF Fe) IS SUFFICIENT TO CAUSE ANEMIA.

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 INGESTION OR  LOSS OF Fe2+ ALTER THE BALANCE IN ITS MET. AND WHEN Fe DEMAND IS GREATER THAN ITS SUPPLY, ANEMIA DEVELOPS IN THREE CHARACTERISTIC STAGES:

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STAGE I: BODY Fe STORAGES ARE DEPLETED BUT ERYTHROPOIESIS AND Hb CONTENT IN RBC REMAIN NORMAL.
 STAGE II: NOT ENOUGH Fe AVAILABLE AND Fe-DEF. ERYTHROPOIESIS BEGINS.
 STAGE III: SMALL, Hb-DEF. CELLS ENTER THE CIRCULATION REPLACING THE OLD NORMAL RBC
REMOVED FROM BLOOD

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IRON DEFICIENCY MANIFESTATIONS AND SIGNS AND SYMPTOMS

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CLINICAL MANIFESTATIONS HAVE A GRADUAL ONSET AND USUALLY ARE
WELL-TOLERATED TILL Hb DROPS
TO  7-8 g/dl.
 
FATIGUE; WEAKNESS; DYSPNEA;
PALE SKIN AND MUCOSE MEMB.;
THIN, BRITTLE AND SPOON-SHAPED OR CONCAVE (KOILONYCHIA) NAILS; GLOSSITIS; GASTRITIS; HEADACHE; IRRITABILITY;
NEUROMUSCULAR CHANGES.

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IRON DEFICIENCY EVALUATION

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BASED ON
CLINICAL MANIFESTATIONS AND
LAB TESTS WHICH MEASURE THE
Fe STORES DIRECTLY
(BONE MARROW BIOPSY) OR
INDIRECTLY
(SERUM FERRITIN,
TRANSFERRIN SATURATION OR
TOTAL IRON-BINDING CAPACITY).
TREATMENT WILL FIND AND ELIMINATE
(OR RULE OUT) ALL SOURCES FOR BLOOD LOSS AND THEN Fe ADM. WILL SOLVE THE PROBLEM SO EFFECTIVELY THAT
AN  IN Hb OF 1-2 g/dl AFTER INITIATION OF Fe THERAPY IS THE MOST CONCLUSIVE DIAGNOSIS

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NORMOCYTIC-NORMOCHROMIC ANEMIAS
 
THESE ANEMIAS ARE LESS COMMON THAN THE PREVIOUS TYPES, DO NOT SHARE ANY COMMON CAUSE AND INCLUDE:

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APLASTIC ANEMIA (don’t produce enough)
--POSTHEMORRHAGE ANEMIA

--HEMOLYTIC ANEMIA (destruction, problems with production or increase in loss of them)
--ANEMIA OF CHRONIC
INFLAMMATION

--SICKLE CELL ANEMIA

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APLASTIC ANEMIA RESULTS FROM

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BONE MARROW HYPOPLASIA OR APLASIA A CONDITION WHERE ERYTHROCYTE STEM CELLS ARE UNDERDEVELOPED, DEFECTIVE OR ABSENT AND WHICH MAY LEAD TO PANCYTOPENIA WHERE ALL THE SERIES ARE AFFECTED
Series is like RBC, WBC, AND PLATELETS. Ionizing radiation. Resulting from external conditions. Infiltration of the bone marrow with abnormal tissue. Like leukemias and prevent production of normal cells.

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APLASTIC ANEMIAS ARE

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HEREDITARY (FANCONI ANEMIA –DEFECT IN DNA REPAIR) AND ACQUIRED WHICH MAY BE:
--PRIMARY (IDIOPATHIC APLASTIC ANEMIA  50% OF CASES WITH 80% OVER
50 YRS. OF AGE).
 --SECONDARY (CAUSED A VARIETY OF CHEMICAL AGENTS AND IONIZING RADIAT). Most frewquent.

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PURE RED CELL APLASIA (PRCA)

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IS VERY RARE AND AFFECTS THE RED SERIES ONLY.
 
CLINICAL MANIFESTATIONS WILL DEPEND OF THE SERIES PREDOMINANTLY AFFECTED AND MAY BE MILD OR SEVERE.

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APLASTIC ANEMIA EVALUATION

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EVALUATION REQUIRES
BONE MARROW BIOPSY AND THE
TREATMENT IS AIMED TO FIND AND TREAT THE UNDERLYING DISORDER OR PREVENT FURTHER EXPOSURE TO CAUSAL AGENT.
PALLIATIVE TREAT. INCLUDES
BLOOD TRANSFUSIONS AND THE FINAL ONE IS MARROW TRANSPLANT OR PHARMACOLOGIC STIMULATION OF THE MARROW FUNCTION.
 
INFECTIONS AND HEMORRHAGE ARE TREATED AS NEEDED AND SPLENECTOMY MAY BE NECESSARY.
Aplastic requires bone marrow biopsy. Most of the time it is from a drug the patient is taking and you need to stop them from taking the offending drug. The spleen is important in immune and lymphatic and the spleen is where all the RBCs are destroyed. (reducing the destruction)

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HEREDITARY SPHEROCYTOSIS

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ALSO KNOWN AS
CONGENITAL HEMOLYTIC ANEMIA OR
ACHOLURIC JAUNDICE IS THE MOST COMMON TYPE OF HEMOLYTIC ANEMIA WITH NORMAL Hb.
 
THE DEFECT IS BELIEVED TO BE CAUSED BY AN UNKNOWN ABNORMALITY OF PROT. IN THE RBC MEMB.   PERMEABILITY TO Na + LEADING TO
ATP CONSUMPTION TO MAINTAIN THE Na-K PUMP WORKING WHICH CAUSES
EARLY AGING AND DESTRUCTION OF RBC (DUE TO METABOLIC OVERWORK AND DEFECTIVE MEMB.) AS WELL AS AN ABNORMAL RBC SHAPE (SPHEROCYTE.
The RBCs are spheres. They rupture and hemolysis. Acholuric means jaundice without hepatic involvement

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CIRCULATION OF SPHEROCYTES THROUGH THE SPLEEN IS STRESSFUL RESULTING IN SEQUESTRATION AND DESTRUCTION

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CLINICAL MANIFESTATIONS STARTING IN THE NEONATAL PERIOD OR EARLY INFANCY INCLUDE ANEMIA AND HYPERBILIRUBINEMIA SO SEVERE THAT USUALLY REQUIRE EXCHANGE TRANSF. OR PHOTOTHERAPY.
AFTER INFANCY, SPLENOMEGALY IS ALMOST ALWAYS PRESENT. GALLSTONES CAN OCCUR AS EARLY AS 4-5 YRS. OF AGE BUT TYPICALLY DEVELOP LATER
(50% OF CASES WITHOUT SPLENECTOMY).
APLASTIC CRISES ARE THE MOST SERIOUS COMP. DURING CHILDHOOD.

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HEREDITARY SPHEROCYTOSIS

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INCLUDES FAMILY HISTORY, BLOOD SMEAR AND STUDIES OF OSMOTIC FRAGILITY AND AUTOHEMOLYSIS BUT THERE’S NO SPECIFIC TEST AND SPLENECTOMY ALWAYS PRODUCES A CLINICAL CURE
(SHOULD BE PERFORMED AFTER 5 YRS. OF AGE).
Clinical cure is the splenectomy. The disease is still there but you won’t have symptoms. Early years the spleen is very important

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SICKLE CELL ANEMIA

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CHARACTERIZED BY THE PRESENCE OF Hb S RESULTING FROM A MUTATION WHICH REPLACES THE GLUTAMIC ACID BY VALINE AND THIS NEW Hb REACTS TO DEOXYGENATION AND DEHYDRATION BY SOLIDIFYING AND STRETCHING THE RBC INTO ELONGATED CRESCENTS WHICH ARE STIFF AND CAN’T CHANGE SHAPES AS THE NORMAL RBC WHEN PASSING THROUGH THE MICROCIRCULATION RESULTING IN A TENDENCY TO OCCLUDE THE BLOOD VESSELS  ISCHEMIA, PAIN AND INFARCTION.
Normal Hgb is Hgb A. Stave off malaria, but then they suffer of this and die just at a later age. Subicteritic conditions. (sclera) internal necrosis. Bone marrow becomes hyperplastic (trying to produce more RBC).
(POIKILOCYTOSIS)

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SICKLE CELL MANIFESTATIONS

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THESE SICKLE CELLS UNDERGO HEMOLYSIS OR SEQUESTRATION IN THE SPLEEN  BLOOD POOLING + INFARCTION OF SPLENIC VESSELS AND  ERYTHROPOIESIS IN THE MARROW AND SOMETIMES THE LIVER.
 
CLINICAL MANIFESTATIONS INCLUDE THOSE COMMON TO ALL
HEMOLYTIC ANEMIAS (PALLOR, JAUNDICE [ICTERUS], FATIGUE AND IRRITABILITY) AND SOMETIMES ACUTE MANIFESTATIONS KNOWN AS CRISES:

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4 TYPES OF SICKLE CELL

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VASOOCCLUSIVE (THROMBOTIC) CRISIS WHICH MAY BE SPONTANEOUS OR PRECIPITATED BY INFECTIONS, EXPOSURE TO COLD,  PO2 ,  pH OR LOCALIZED HYPOXEMIA.
APLASTIC CRISIS RESULTS FROM RBC WITH SHORTER SURVIVAL (10-20 DAYS) AND A COMPROMISED COMPENSATORY RESPONSE. SEQUESTRATION CRISIS IN BOTH THE SPLEEN AND LIVER. (and sinusoids occluded and destroyed)

HYPERHEMOLYTIC CRISIS IS UNUSUAL BUT MAY OCCUR IN ASSOCIATION WITH CERTAIN DRUGS AND INFECTIONS.

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SICKLE CELL MANIFESTATIONS

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INFECTION IS THE MOST FREQUENT CAUSE OF DEATH (MENINGITIS AND GENERAL SEPSIS)
EVALUATION INCLUDES THE
PARENT’S HEMATOLOGIC HISTORY AND ANALYSIS OF CLINICAL MANIFEST. BUT HEMAT. TEST ARE INDISPENSABLE.

WHEN SICKLE CELL SOLUBILTY TEST CONFIRMS THE PRESENCE OF HbS IN PERIPHERAL BLOOD,
Hb ELECTROPHORESIS INFORMS ITS AMT. IN THE RBC.
PRENATAL DIAG. CAN BE MADE WITH CHORIONIC VILLUS SAMPLE
( 8-10 WEEKS’ GESTATION) OR AMNIOCENTESIS (15 WEEKS’ GESTATION).
Basic treatment is transfusion of RBC. Only available important treatment. Other than that you just treat the symptoms. Genetic counseling. A way to prevent the disease

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TREATMENT OF SICKLE CELL

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TREATMENT IS AIMED TO
PREVENT CONSEQUENCES OF THE ANEMIA AND PREVENT CRISES
(AVOIDING FEVER, INFECTION, DEHYDRATION, ACIDOSIS, EXPOSURE TO COLD).
BONE MARROW TRANSPLANTATION HAS BEEN SUCCESSFUL IN PAT. WITH CONCOMITANT ACUTE LEUKEMIA.
BLOOD TRANSFUSION CAN BE VERY EFFECTIVE BUT IT MUST BE EVALUATED DUE TO THE RISKS (HEPATITIS, HIV, HEMOSIDEROSIS).
ORAL ADM. OF FOLATE AND SPLENECTOMY IF NECESSARY ARE ALSO INDICATED.
GENETIC COUNSELING IS VITAL BECAUSE THERE’S A 25% CHANCE WITH EACH PREGNANCY THAT A CHILD BORN TO TWO PARENTS WITH THE TRAIT WILL HAVE THE DIS.

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THALASSEMIAS (sea water) greek people

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CHARACTERIZED BY THE IMPAIRED RATE OF SYNTHESIS OF ONE OF THE  OR  CHAINS   OR  THALASSEMIA.

DEPENDING OF THE DEGREE OF DEPRESSION IN THE CHAIN PRODUCTION
THEY’RE ALSO CLASSIFIED IN MINOR OR MAJOR.
BOTH  AND  THALASSEMIA MAJOR ARE LIFE THREATENING WITH CHILDREN WHO ARE WEAK, FAIL TO THRIVE,
SHOW POOR DEVELOPMENT AND PRESENT CARDIOV. COMPROMISE
(HIGH-OUTPUT HEART FAILURE SECONDARY TO ANEMIA).

WITHOUT TREATMENT PAT. WILL DIE AT
5-6 YRS. OF AGE.

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THALASSEMIA TREATMENT AND EVALUATION

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EVALUATION IS BASED ON
FAMILY HISTORY, CLINICAL MANIFEST., PERIPHERAL BLOOD SMEARS (MICROCYTOSIS) AND
Hb ELECTROPHORESIS.
 
TREATMENT INCLUDES
BLOOD TRANSFUSIONS,
IRON CHELATION THERAPY (EXPERIMENTAL),
SPLENECTOMY AND
NEOCYTE TRANSFUSION (EXPERIMENTAL)..

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POLYCYTHEMIAS

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CONSISTS ON AN EXCESSIVE PRODUCTION OF RBC
 
THE RELATIVE TYPE RESULTS FROM HEMOCONCENTRATION OF THE BLOOD USUALLY DUE TO DEHYDRATION AND THE ABSOLUTE NUMBERS OF RBC ARE NORMAL AS WELL AS THE MARROW FUNCTION.
 
THE ABSOLUTE TYPE INCLUDES THE PRIMARY AND THE SECONDARY.