hgb/hctx100

15/47x100=31.9

31.9%

A decreased osmotic fragility test would be associated with which of the following conditions?

(less fragile)

Sickle cell anemia. (decreased) it would be increased in sperocytosis.

(more fragile)

Intravascular hemolysis

 Classic features of intravascular hemolysis such as hemoglobinemia, hemoglobinuria or hemosiderinuria do not occur in heriditaty spherocytosis. The hemolysis in heriditary spherocytosis is extra vascular rather than intravascular.

Intra vascular hemolysis.

Classic features of intravascular hemolysis such as hemoglobinemia, hemoglobin uria, or hemosiderin uria, do not occur in heriditary spherecytosis. The hemolysis seem in heriditary spherocytosis is an extravascular rather than intravascular process.

Heriditary spherocytosis.

Spherocytic cells have decreased tolerance to swelling and , therefore hemolyze at a higher concentration of sodium salt compared with normal red cells.

reticulocytopenia.

The hemolytic anemia of HUS is associated with reticulocytosis. The anemia seen in HUS is multifactorial, with characteristic schistocytes and polychromasia commensurate with the anemia.

Paroxysmal nocturnal hemoglobinuria. (PNH)

The autohemolysis test is positive in G6PD and PK deficiencies and in HS but not in PNH because lysis in PNH requires sucrose to enhanse compliment binding. The addition of glucose, sucrose, or adenosine triphophate (ATP) corrects the Autohemolysis of HS. Autohemolysis of PK can be corrected by ATP.

Anti-P

PCH is caused by the anti-P antibody, a cold autoantobody that binds to the Patients RBCs a low tempertures and fixes complement, In the classic Donath-Landsteiner test, hemolysis is demonstrated in a sample placed at 4°C that is then warmed to 37°C.

Increased Haptoglobin.

Haptoglobin is a protein that binds to free Hgb. The increased free Hgb in intravascilar hemolysis causes depletion of haptoglobin.

Spherocytic red cells.

Spherocytes are charactistic of autoimmune hemolytic anemia and result in an increased osmotic fragility. In autoimmune hemolytic anemias (AIHAs), production of auto antibodies aginst ones own red cells causes hemolysis or phagocytic destruction of RBCs . A Positive direct antiglobulin (DAT or coombs) test identifies in vivo antibody-coated and compliment coated red cells. A positive DAT distinguishes AIHA from other types of hemolytic anemia that produce spherocytes. Those AIHAs that produce complement binding antibodies cause a depletion of C3. Those not associated with intravascular hemolysis cause an increase in unconjugated biliruben 

G6PD deficiency.

In patients with G6PD deficiency the red cells are unable to reduce nicotinamide adenine dinucleotide phosphate (NADP) to NADPH; consequently, Hgb is denatured and Heinz bodies are formed. "Bite cells" appear in the peripheral circulation as a result of splenic pitting of heinz bodies.

RBC indices.

RBC indices classify the anemia morphologically Anemias can be classified morphologically by the use of laboratory data, physiologically based upon the mechanism, and clinically based upon an assesment of symptoms.

Peripheral blood pancytopenia.

Aplastic anemia has many causes, such as chemical, drug or radiation poisoning, congenital aplaysia and fanconis syndrome. All result in depletion of hematopoietic precursors of all cell lines. leading to peripheral blood pancytopenia.

Bizarre multinucleated erythroblast.

There are four classifications of (CDAs) each characterized by inneffective erythropoisis, increased unconjugated biliruben, and bizarre muitnucleated erythroid precursors.

Schistocytes and nucleated RBCs.

Microangiopathic hemolytic anemia is a condition resulting from shear stress to the erythrocytes. Fibrin strands are laid down within the microcirculation and red cells become fragmented as they contact fibrin through the circulaton process.

Chloramphenocol

Chloramphenocol is a drug most often implicated in aquired aplastic anemia. Almost half of the cases occur within 30 days after therapy and about half of the cases are reversible.

Heriditary intracorpuscular RBC defects.

Sickle cell disorders are intracorpuscular red cell defects that are heriditary and result in defective Hgb being produced. The gene for sickle cell can be  inherited either homozygously or heterozygously.

Increased MCV.

This patients abnormal peripheral smear indicates marked red cell regeneration, causing many reticulocytes to be released from the marrow. Since reticulocytes are larger than mature RBCs, the MCV will be slightly elevated.

Howell Jolly bodies.

As a result of a splenectomy, Howell Jolly bodies may be seen in great numbers. One of the main functions of the spleen is the pitting function, which allows inclusions to be removed from the red cell without destroying the cell membrane.

Red cell regeneration.

Reticulocytes are polychromatophillic macrocytes, and the presence of reticulocytes indicates red cell regeneration. The bone marrows appropriate response to anemia is to deliver cells prematurely to the peripheral circulation. In this way, reticulocytes and possibly nucleacted red cells may be seen in the perpheral smear.

Misshapen budding fragmented cells.

HP is a membrane defect characterized by a spectrin abnormaliy and thermal instability. The MCV is decreased and the red cells appear to be budding and fragmented.

Decreased.

Numerous target cells are persent in tallassemia major patients. Because target cells have increased surface volume , the osmotic fragility is decreased.

Decreased total iron-binding capacity.

There is an increase in TIBC and RBC protoporphrin because of a decreased level of iron in iron deficicency anemia. Morphological characteristics  of iron deficicency anemia include microcytic . hypochromic blood picture. Platelets are usually small and increased in number.

Total iron binding capacity.

In iron deficency anemia the serum iron level is decreased.  In chronic disease the iron is trapped in reticuloendothelial cells (RE) and therefore is unavailable to the red cells. serum iron and TIBC are both decreased.

Thallasemia syndrome.

Iron deficicency anemia and thalassemia are both classified as microcytic, hypochromic anemias. iron deficicency anemia is caused by defective heme synthesis, whereas thalassemia is caused by decreased globin chain synthesis.

Serum ferritin levels

Ferritin enters the system from all ferritin producing tissues and therefore is considered to be a good indicator of body storage iron. Because iron stores must be depleted before anemia develops, low serum ferritin levels  precede the fall in serum iron associated with iron deficency anemia.

Increased RBC protoporyphrin;

Sideroblastic anemia has a decreased red cell protoporphryn. The defect in sideroblastic anemia involves ineffective erthropoiesis . The failure to produce RBC protoporphryn occurs because the non-heme iron is trapped in the mitochondria and is un-available to be recycled.

Beta chain synthesis.

In thalassemia Major there is little or no prodction of the beta chain , resulting in severly depressed or no systhesis of Hgb A . Severe anemia is seen, along with skeletal abnormalities and marked splenomegaly.The patient is usually suported with transfusion therapy.

Hgb F.

Patients with thallasemia major are unable to synthesize the Beta chain; hence little or no hgb A is produced. However gamma chains continue to be synthesized and lead to variable elevations of Hgb F in these patients.

Microcitic hypochromic.

The indices will provide a morphological classification of this anemia. The MCV is 75. (80-96), MCH is 20 (27-33)and the MCHC is 26.(33-36) therefore , microcytic hypochromic.

Beta thal minor.

Hgb A₂ is part of the normal complement of adult Hgb. This Hgb is elevated in Beta-thalassemia minor because the individual with this condition has only one normal B gene consequently there is a higher elevation of Hgb A₂ and HgbF.

Decreased serum iron concentrations.

thirty to fifty percent of the individuals with the anemia of chronic inflammation demonstrate a microcytic hypochromic blood picture with decreased serum iron. Serum Iron is decreased beause  it is unable to escape from the RE cells to be delivered to the pronormoblast in the bone marrow.

Macrocytic normochromic

Megaloblastic macrocytic anemia is noromochromic because there is no defect in the hemoglobin systhesis. These anemias are a group of asyncronized anemias characterized by drfective nuclear maturation due to defective deoxyribonucleaic acid (DNA) synthesis. This abnormality accounts for the megaloblastic. features in the bone marrow and the macrocytosis in the peripheral blood.

Pernicious anemia.

Pernicious anemia is caused by a lack of intrinsic factor, which prevents vitamin B₁₂ absorbtion. An abnormal excreation in part 1 indicates that vitamin B₁₂ was not absorbed through the intestine , Normal excreation of labeled B₁₂ after administration of intrinsic factor in Part 2 of the schlling test indicates pernicious anemia

An improvement in hematological abnormalities.

Administration of folic acid to a patient with vitamin B₁₂ deficicency will improve the hemotological abnormalities ; however , the neuralogical  problems will continue. This helps to confirm the correct diagnosis  of vitamin B₁₂ drficicency.

All of the following are characteristice of megaloblastic anemias except:

A.) pancytopenia

B.) Elevated reticulocyte count.

C.) Hypersegmented nutrophols

D.) Macrocytic erythrocyte indices

Elevated reticulocyte count.

Megaloblastic anemias are associated with an ineffective erythropoiesis and therefore a decrease in the reticulocuyte count.

An improvement in hematological abnormalities.

Administration of folic acid to a patient with vitamine B deficiency will improve the hematological abnormalities; it is however, the neurological problems will continue, This helps to confirm the correct diagnosis of vitamone B 12 deficiency. 

Which of the following disoders below causes ineffective erythropoiesis ?

A.) G6PD deficiency

B.) Liver disease

C.) Hgb C disease

D.) Pernicious anemia

D, Pernicious anemia

Ineffective erythropoiesis is caused by destruction of erythroid precursor cells prior to their release from the bone marrow. Pernicious anemia results from defective DNA synthesis, it is suggested that the asyncronous development of red cells renders them more liable to intramedullary destruction.

A 50 year old patient is suffering from pernicious anemia. Which of the following laboratory data are most likely for this patient.?

RBC = 2.5

WBC = 2500

PLT= 50,000

Patients with pernicious anemia demonstrate a pancytopenia with a low WBC, PLT and RBC counts. Because this is a megaloblastic process and a DNA maturation defect, all the cell lines are affected. In the bone marrow this results in abnormally large precursor cells, Maturation asynchrony, hyperplasia  of all cell lines, and a low M:E ratio.

Which of the following may be seen in the peripherial blood smear of a patient with obstructive liver disease?

A.) Schistocytes

B.) Macrocytes

C.) Howell-jolly bodies

D.) Microcytes

D. Macrocytes

Patients with obstructive liver disease may have red blood cells that have an increased tendency toward the deposition of lipid on the surface of the red cell. Consequently, the red cells are larger or more macrocytic than normal red cells.

The macrocytes typically seen in megaloblastic processes are:

Ovalocytic

Macrocytes in true megaloblastic conditions are oval macrocytes as opposed to the round macrocytes that are usually seen in alcoholism and obstructive liver disease.

MCV 125 (big)

MCH 36 (normal)

MCHC 30 (normal)

The red cell indicies in a patient with megaloblastic anemia are macrocytic and normochromic. The macrocytosis is prominent, with an MCV ranging from 100 to 130.

Increased MCV

The patient will have an increased MCV. ne of the causes of a macrocytic anemia that is not megaloblastic is an increased reticulocyte count, here noted as increased polychromasia. Reticulocytes are polychromatic macrocytes; therefore, the MCV is slightly oncreased.

Hemolytic anemia

Occasionally patients with infectious mononucleosis develop a potent cold agglutinin with anti-I specificity. This cold autoantibody can cause strong hemolysis and a hemolytic anemia.

Reactive lymphocytes

HIV infection brings about several hematological abnormalities seen on periphral smear examinaton; most patients demonstratereactive lymphocytes and have granulocytopenia.

Dohle bodies

Dohle bodies are  RNA rich areas within polymorphonuclear neutrophils (PMNs) that are oval and light blue in color . Although often assiciated with infectious states, they are seen in a wide range of conditionsnand toxic reactions, including hemolytic  and pernicious anemias, chronic granulocytic leukemia, and therapy with antineoplastic drugs.

Myeloperoxidase

Myeloperoxidase, lysosome, and acid phosphatase are enzymes that are contained in the primary granules of neutrophils. The contents of secondary and tertiary granules include lactoferrin , collagenase, NADPH oxidase, and alkaline phosphatae.

50-70%

Pelgeroid hyposegmentation.

Neutrophil changes associated with infection may include neutrophilia, shift to the left, toxic granulation, Dohle bodies, and vacuolization. 

Giant lysosome granules.

Chediak-higashi syndrome is a disorder of neutrophil phagocytic dysfunction caused by depressed chemotaxis and delayed degranulation . The degranulation disturbance is attributed to interference from the giant lysosomal granules.

CMV-IgM positive

If both the heterophile antibody test and the EBV-IgM test are negative in a patient with reactive lymphocytosis and a suspected viral infection, the serum should be analyzed for IgM antibodies to CMV

Hydrogen peroxide and superoxide anion.

The biochemical products of the  respiratory burst that are involved with neutrophil particle ingestion during phagocytosis are hydrogen peroxide and superoxide anion. The activated neutrophil discharges the enzyme NADPH oxidase into the phagolysosome, where it converts O2 to superoxide anion, which is then reduced to hydrogen peroxide.

High nuclear:cytoplasm ratio, prominent nucleoli, basophilic cytoplasm

Both reactive lymphocytes and blast may have basophilic cytoplasm, a high N:C ratio, and the presencr of prominent nucleoli

lymphoblast

Auer rods are not seen characteristically in lymphoblasts. They may be seen in myeloblast promyelocytes, and monoblast.

Normochromic, normocytic.

Acute leukemia is usually associated with a normocytic , normochromic anemia.Anemia in acute leukemia is usually present from the onset and may be severe; however there is no inherant nutritional deficicency leading to either microcytic, hypochromic , or megaloblastic process.

Leukoerythroblastosis.

The presence of immature leukocytes and nucleated red cells is denoted leukoerythroblastosis. Myelophthisis refers to replacement of bone marrow by a disease process such as neoplasm. The development of abmnormal tissue is called dysplasia

Decreased erythropoietin production.

What it does

replacement of normal marrow precursors by leukemic cells causing anemia.

Decrease in functional leukocytes causing infection.

Hemorrage secondary to thrombocytopenia.

Acute monocytic leukemia

Acute monocytic leukemia has an incidence of between 1% and 8% of all leukemisa. It has a distinctive clinical manifestation of monocytic involvement resulting in skin and gum hyperplasia. The WBC count is markedly elevated and prognosis is poor.

1-15 years

Acute lymphoblastic leukemia (ALL) usually affects children from the ages of 1-15 and is the most common type of acute leukemia in this age group. In addition ALL constitutes the single most prevalent malignancy in pediatric patients.

leukemia

A disproportionate increase in the myeloid component of the bone marrow is usually the result of a leukemic state. The normal M;E ratio is approximately 1.5-3.0

Acute mylecytic leukemia

AML blasts stain positive for Sudan black B and peroxidase. Usually fewer than 10% blast are found on peripheral smear of patients with CML, unless there  has been a transition to blast crisis. The organelles in the cells of AUL are not mature enough to stain positive fpr SBB or Px. Blasts in ALL are characteristicall negative with these stains.

Sudan black B

Phospholipids, neutral fats , and sterols are stained by Sudan black B. The PAS reaction stains intracellular glycogen . Myeloperoxidase is an enzyme present in the primary granules of myeloid cells and to a lesser degree in monocytic cells. Terminal deoxynucleotidyl transferase(TdT) is a DNA polymerase found in thymus- derived and some bone marrow- derived lymphocytes. 

monocytes

NASDA stains monocytes and monoblast. The addition of fluoride renders the monocytic cells and blasts negative , thus allowing for differentiation from the granulocytic cells which remain positive.

Common ALL

The majority of non-T, non-B ALL blast cells display the common ALL antigen (CALLA) marker. Lymphoblast of common ALL are TdT positive and CALLA positive  but do not have surface membrane IgM or M chains and are pre-B lymphoblast. Common ALL has a lower relapse rate and better prognosis than other immunological subtypes of B cell ALL

Terminal deoxynucleotidyl transferase and PAS

 PAS is positive in about 50% of ALL with L1 and L2 morphology but is negative in ALL with L3 morpholoy (B-cell ALL)  Terminal deoxynucleotidyl transferase is a positivein all types of ALL except L3. Both  Terminal deoxynucleotidyl transferase and PAS are negative in AML.

Acute lymphocytic leukemia

Sudan black B stains phospholipids and other neutral fats. It is the most sensitive stain for granulocytic precursors. Lymphoid cells rarely stain positive for it. 70% lymphoblast would never be seen in CLL,

cytogenic abnormalities

 In addition to morphology , cytochemical stains and flow cytometry, The who classification relies on chromasomal and molecular abnormalities.

Four subtypes with commonly occuring translocatons encompassmost cases of AML.

Iron deficiency anemia

Iron deficiency anemia is a predictable

cpmplication of theraputic phlebotomy because approximately 250 mg of iron is removed with each unit of blood

In essential thrombocytopenia the platelets are:

Schistocytes.

The marked amount of fibrosis, both medullary and extramedullary, accounts for the irreversible red cell morphological change to a teardrop shape. The red cells are teared as they attempt to pass through the fibrotic tissue

Splenomegally

splenomegaly is a feature of PV but not characteristic of secondary polycythemia. Erythropoietin is increased and oxygen  and oxygen saturation  is decreased in secondary  polycythemia

The leukocyte alkaline phophatase (LAP) stain on a patient gives the following results:

10(0), 48(1+), 38(2+),3(3+),1(4+) calculate the LAP score