1. porphobilinogen

   2. uroporphyrinogen

     3. coproporphyrinoge

       4. protoporphyrinogen

hemoglobin

two-thirds

[image]

Which curve represents the production of alpha polypeptide chains of hemoglobin

starts and peaks soon after fetal development persist throughout life

hemoglobinA

Which curve represents the production of beta polypeptide chains of hemoglobin?

[image]

Which curve represents the production of gamma polypeptide chains of hemoglobin

[image]

Delta begins at about 6-7 months gestation and is nonexistant by four months old

Delta never climbs above 1 or 2 % of total hemoglobin

Which curve represents the production of epsilon polypeptide chains of hemoglobin

[image]

In the ferrous state

(reduced)

myeloid metaplasia

(myelofibrosis)

Macrocytic

impaired DNA synthesis, asyncrounous development

denatured hemoglobin inclusions that are readily removed by the spleen

Formed with oxidation of Fe and formation of methhemoglobin

caused by a red cell membrane defect.

RBC mebrane defect (DAF) increases susceptibility to complement mediated lysis.

spherocytes

May be referred to as hyperchromic becaused oof increased MCHC

iron

iron loss with loss od blood

decreased serum iron levels

Serum Fe levels low due to sequestration in macrophages and hepatocytes.

aplastic crisis

this is common in sickle cell disease

A 89 year old Caucasian female was transferred to the hospital from a nursing facility for treatment of chronic urinay tract infection with proteinuria. The patient presented with the following laboratory data:

WBC: 10.0, RBC 3.1,  Hgb 7.2,  Hct 24%,  MCV,  78,  MCH 23,  MCHC 31%,  serum iron 29ug/dL,  TIBC 160  serum ferritin 100

These data are most consistant with which of the following conditions? 

A patient is admitted with a history of chronic bleeding secondary to peptic uncer. Hemotology workup reveals a severe microcytic, hypochromic anemia,. Iron studies were requested. Which of the following would be expected in this case?

             Serum iron               TIBC          Storage iron

result A     decreased             increased        increased

result B      increased              decreased        increased 

result C      decreased            increased         decreased

result D     increased              normal              decreased   

C

Iron studies: microcytic hypocromic anemia results in a decrease in serum and storage iron, but an increase in TIBC.

thalassemia minor

FEP thalassemia

normocytic, normochromic

it is the number of RBC that is decreased

Which of the following sets of laboratory findings is consistant with hemolytic anemia?

A. normal or slightly increased erythrocyte survival; normal osmotic fragility.

B. decreased erythrocyte survival; increased catabolism of heme.

C. decreased serum lactate dehydrogenase activity; normal catabolism of heme

D. normal concentration of haptoglobin; marked hemoglobinemia:

spontaneously

G6PD deficency - hemolytic stimulus

Vitimine B12 deficency

(pancytopenia, macrocytosis)

A patient has the following laboratory data

RBC: 2.35

WBC 3.0

Plt: 95.0

Hgb: 9.5

Hct: 27%

MCV: 115 (high)

MCHC: 35%(high)

MCH: 40

Which of the following test would contribute to this diagnosis?

A 50 year old patient was found to have the following Lab results:

Hgb: 7.0

Hct: 20%

RBC: 2.0

It was determined that the patient was suffering from pernicious anemia. Which of the following sets of results most likely was obtained from the same patient:

                      WBCs            Platelets             Reticulocytes 

result A          17,500          350,000             5.2%

result B          7,500            80,000               4.1%

result C          5,000           425,000               2.9%

result D           3,500          80,000                 0.8%

Result D

Macrocytosis and Pancytopenia

are the classic findings for pernicious anemia

neutropenia and thrombocytopenia.

Pancytopenia

Which of the following represents characteristic features of iron metabolism in patients with anemia of chronic disease

                          Serun iron    Transferrin saturation  TIBC

result A              normal            normal                    normal

result B               increased       increased                nor/sl inc

result C               normal          marked inc               normal

result D               decreased      decreased               nor/decre   

result D

Iron studies in anemia of chronic disease show decreased serum iron and transferrin saturation; TIBC may be decreased as well.

A patient has the following blood values:

RBC 6.5

Hgb: 13.0

Hct: 39.0%

MCV: 65

MCH: 21.5

MCHC: 33%

these results are compatible with:

Thalassemia minor

normochromic, noromocytic

decreased osmotic fragility

They do include:

increased auto-hemolysis corrected by glucose

reticulocytosis

shortend erythrocyte survival

A 14 year old boy is seen in the ER complaining of a sore throat, swollen glands and fatigue. The CBC results are:

WBC 16.0

RBC: 4.37

Hgb: 12.8

Hct: 38%

Plt: 180

Differential:

absulote neutrophils:   3.9

absolute lymphs: 6.0

absolute monos: 0.5

absolute atypical lymphs: 3.2 

What is the most likely diagnosis?

infectious mononucleosis

In infectiuos mononucleosis, the leukocyte count is usually increased due to an absolute lymphocytosis. The platelet count is often mildy decreased. 

small fibrin clots in the sample

Also when the shape or size of the red blood cells prevents rouleaux formation a decreased or low ESR is expected. This is observed with sickle cells, acanthocytes and shherocytes.

A wright stained peripheral smear reveals the following:

Erythrocytes enlarged 1 1/2 to 2 X normal size

Schuffner dots

Parasites with irregular "spreadout" trophozoites, golden brown pigment.

12-24 merozoites

Wide range of stages.

This is consistant with Plasmodium:

Vivax

24 karet gold

Using a supra vital stain, the polychromatic red cell below would probably be :

[image]

Vitamine B12 deficiency

pancytopenia and macrocytes

5.0%

Newborn infants have elevated reticulocyte counts.

The following results were obtained on a patients blood;

Hgb: 11.5

Hct:  40%

MCV: 26

MCH: 89

MCH: 26

MCHC: 29%

Examination of a wrights stained smear of the sampe same sample would most likely show:

basophillic stippling, nuecleated red blood cells and polychromasia

Polychromatic red blood cells and basophillic stippling can be associated with accelerated heme synthesis.

The smear below displays:

 [image]

sedimentation rate

Rouleaux and an increased sedimentation rate are caused by increased fibrinogen and/or with increased immunoglobulin. 

elevated

The national Polycythemia Vera study group criteria for the diagnosis of polycythemia vera include increased red cell mass, increased platelet count, increased LAP score.

extensive burns

Patients who have suffered severe burns to more than 15% of their body generally show evidence of intervascular hemolysis, RBCs show changes including fragmentation, budding and microspherocytes formation.

The values below were obtained on a aautomated blood count system performed on a blood sample from a 25 year old man:

            Patient                    Normal

WBC      5.1                         5.0 -10.0   

RBC        2.94                      4.6-6.2

Hgb:        13.8                        14-18

Hct:        35.4                        40-50%

MCV        128                        80-90

MCH       46.7                         27-31   

MCHC:    40%                        32-36

These results are most consistant with which of the following?  

 a high titer of cold agglutinins

erroneously elevated MCV and erronously decreased RBC count(due to clumping of cells)

A 56 year old man was admitted to the hospital for treatment of a bleeding ulcer. The following laboratory data was obtained.

RBC   4.2

WBC:   5.0

Hct:      30%

serum iron : 40ug/dL

TIBC:  460

serum ferritin 12

Examination of the bone marrow revealed the absence of iron stores. This data is most consistant with wich of the following conditions?

iron deficiency anemia

Laboratory findings in iron deficiency anemia include decreased serum iron, serum ferritin, % saturation, and increased TIBC, FEP, and serum soluble transferrin receptor levels.

A fourty year old caucasian male was admitted to the hospital fro treatment of anemia, lassitude, weight loss, and loss of llibido. The patient presented with the folllowing laboratory data:

WBC:    5.8

RBC:     3.7

Hgb:     32%

MCV:     86

MCHC:     32%

serum iron:    220

TIBC:        300

serum ferritin:     2,800

Examination of the bone arrow revealed erythroid hyperplasia with a shift to the left of erythroid precursers. Prussian blue staining revealed markedly elevated iron stores noted with occasional sideroblast seen. This is most consistant with which of the following conditions.

hemochromotosis

Secondary hemochromotosis is associated with anemia with abnormaly high serum iron levels.

very high WBC count

Turbidity in cyanmethemoglobin reagent- patient specimen will result in falsely elevated hemoglobin values.

elevated A2 hemoglobin

Hemoblobin A2 is increased in beta thalassemia, but decreased in alpha thal

With this blood picture, an additional test indicated is:

[image]

hemoglobin electrophoresis

Hgb C crystals can be seen in patients with Hgb C disease, more often in individuals more often in individuals who have undergone splenectomy.

Kleihauer-Betke

The Kleihauer-Betke procedure is commonly used as a screening test to determine the amount of fetal blood that has mixed with maternal blood.

dithionite soluability

The soluability test is a rapid test for Hgb S, This should not be used for screening newborns, needs to be corrected in severe anemia and is not specific for Hgb S as there are other hemoglobins that will sickle. The presence of Hgb S is confirmed by hemoglobin electrophoresis.

Hgb F.

In delta-betthalassemia, one or both of the gamma genes remain, resulting in 100%F.

its oxygen affinity is lower than that of hemoglobin A

Hemoglobin H does show increased oxygen affinity.

gamma chain production equals alpha chain production

In the black and swiss types of HPFH, both gamma and alpha chains are produced in approximately equal amounts.

increased hemoglobin

The low hemoglobin can result in a false negative result.

A native of thiland has a normal hemoglobin lavel. Hemoglobin electrophoresis on cellulose acetate shows 70% hemoglobin A and approximately 30% of a hemoglobin(E+A2) with the mobility of hemoglobin A2 . This is most consistant with hemoglobin:

The Laboratory findings on a patient are as follows :

MCV: 55

MCHC: 25%

MCH: 17:

A stained blood film of this patient would most likely reveal a red cell picture that is: 

A patient has the following laboratory results:

RBC:  2.00

Hct:  24%

Hgb:  6.8

Reticulocyte count: 0.8%

The mean corpuscular volume (MCV) of the patient is:

24 x 10/2.00 = 120

MCV = 120

The following results were obtained from a post surgical patient recieving total paranteral nutrition:

Hospital day    17                    18                          19

Hgb                 12.1                 11.6                        9.4

Hct                  29.2                 29.4                       28.8

The most consistant explaination for the above data is:    

(5x10)/2.4

MCH = 21

(6X 10)/ 1.5

MCH= 40

6 X100/20

MCHC= 30

Given the following data

Hgb:  8

Hct:   28%

RBC:  3.6

The MCV is: 

28X10/3.6

MVC= 83

20x10/1.5

MCV= 133

Serum iron   decreased

Serum TIBC increased

% saturation   decreased

Marrow % sideroblast   decreased

marrow iron stores   decreased

Serum ferritin    decreased

Hgb A2    decreased

Inclusions in the cytoplasm of neutrophils as shown in the figure below are known as:

[image]

Phagcytosis is a function of:

a shift in granulocytes from the marginating pool to the circulation pool

Glucorticoid induced granulocytosis.

megaloblastic anemia

Differentiates anemia due to conditioned nutritional deficiency from those of bone marrow dysfunction

The following results were obtained on a 45 year old man complaining of chills and fever:

WBC: 23.0

LAP: 200

differential

Segs 60%

bands 21%

lymphs 11%

monos 3%

metas 2%

myelos 3%

Toxic granulation, Dohle bodies and vacuoles

These e=results are consistant with  

neutrophilic leukemoid reacton

Lab findings , increased WBC but with increased LAP; negative philadelphia chromosome differentiates leukamoid response from leukemia.

lymphocytes

predominant cell line in viral response is lymphocytes

cytomegalovirus (CMV) infection

differentiates CMV as most common of viral diseases that lack serological evidence of infection. 

Auer bodies are:

granulocytic hyperplasia

(normal is 3:1 or 4:1)

The following results were obtained:

WBC:   5.0

RBC:   1.7

MCV:   84

Plt:        89

LAP:   142

Philadelphia chromosome: negative

Differential :

Segs 16%

Bands:   22%

lymphs:   28%

Monos:  16%

Eos:   1%

Basos: 1%

Metamyelocytes:   4%

Myelos:   31%

Promyelos:   4%

Blasts:   5%

1 megakaryoblast; 30 nucleated erythrocytes; teardrops; schistocytes; polychromasia.

This is consistant with

Leukoerythroblastosis in mylofibrosis.

Peripheral findings demonstrate condition as mutliplasia, and exclude single cell line disease.

A 50 year old man was admitted into the hospital with acute leukemia. Laboratory findings included the following:

Myeloperoxidase stain:     blast cells negative

PAS stain:                         blast cells demonstrate a blocking pattern

TdT:                                  blast cells positive

Surface immunoglobulin:  blast cells negative

CD2:                                  blast cells negative

Philadelphia chromosome:  Positive

These results are consistant with:

Chronic myelogenous leukemia in lymphoblastic transformation.

results demonstrate a lymphoblastic cell line and 1/3 of cases od CGL in blast phase are consistant with ALL.

A 30 year old man who had been diagnosed as having leukemia 2 years perviously was readmitted because of cervical lymphadenopathy. Laboratory findings included the following:

WBC:   39.6

RBC:    3.25

Hgb:      9.4

Hct:       28.2%

MCV:    29.0

MCH:    29

MCHC:  33.4%

Plt:         53

LAP:      11

Philadelphia chromosime: positive

Differential:

Polys:       7%

Lymphs:   4%

Monos:     2%

Eos:          3%

Basos:       48%

Myelos:     13%

Promyelos   2%

metamyelos: 8%

Blasts:           13%

NRBCs :         11

Bone Marrow:   95% cellularity, 50% blast cells (some with peroxidase and SBB positivity)

These results are most consistant with:

CML in blast transmation.

>20% Blast in peripheral or marrow defines blast conversion. Lab results with % precursor and blast identify progression of this patient from chronic to acute phase.

The following results were obtained on a 35 year old woman complaining of fatigue and weight loss:

WBC:   1.8 

RBC:     4.6

Plt:        903

Uric acid 6.4

LAP         0

Philadelphia chromosome:    Positive

Differential:  

Segs:         30%

Bands:       17%

Lymphs:    13%

Monos:        3%

Eos:              4%

Basos:           6%

Metamyelocytes:  3%

Myelos:         20%

Promyelos:   3%

Blasts:           1%

These tesults are consistant with:

chronic myelocytic leukemia:

Leukopenia with immature granulocytes in all stages, including blast with markedly decreased LAP and Philadelphia Chromosome, positively identify CML. Leukopenia is consistant with engorged marrow space.

Acute myelomonocytic leukemia.

Demonstrates the progression of multiple myeloma

increased serum B12 binding capacity

Lab findings are reflective of erythroid rather than myeloid metaplasia

acute myelocytic leukemia

>20% of myeloblast without other immature stages differentiates AML from CML amd myeloid metaplasia; erythroleukemia requires at least 50% erythroid precursors in the marrow.

Disseminated intervascular coagulation.

These findings differentiate PML from sideroblasitc anemia, mylefibrosis and CGL

lysozymuria

leukemic hiatus

definition of haitus as opening or breaks demonstrates the absence of intermediate maturing cells

Philadelphia chromosome

Classic decrimination between CGL vs other hematalogical conditions: sideroblastic anemia, PML, mylefibrosis.

Acute myelocytic leukemia.

Acute myelogenous leukemia

Acute leukemia is characterized by a maturation defect, whereby immature hemetopoietic progenitors connot overcome a block in differentation, also known as the leukemic haiatus.

chronic myelocytic leukemia

Demonstrates difference between acute vs chronic leukemias; intermediate cell maturity seen in chronic myelogenous , but would not be predominate in erythroleukemia. 

polycythemia Vera:

tell the difference between granulocytic precursor, which would exclude lymphocytic leukemia

The following results were obtained on a 55 year old man complaining of headache and blurred vision:

WBC:   19.0

RBC:     7.2

Plt:         1,056

Uric acid: 13.0

O2 saturation:  93%

Red cel volume:   3,911

Differential

Segs:      84%

Bands:    10%

Lymphs: 3%

Monos:    2%

Eos:         1%

These results are consistant with

Polycythemia vera

results reflect polycythemia vera's excessive proliferation of multiphasic cell lines (megakaryocytes, erythroid and myelocytoid).

polycythemia associated with renal disease

erythopoitetin is made in the kidney.

phlebotomy.

hypochromic, microcytic RBC is most commonly associated with iron deficency, which would most likely result from repeated theraputic phlebotomies

A patient has been treated for polycythemia vera for several years. His blood smear shows:

Oval macrocytes

Howell-jolly bodies

Hypersehmented neutrophils

Large, agranular platelets

The most probable cause of this blood picture is:

chemotherapy

smear findings demonstrate drug impact: megalastic changes due to interference with DNA synthesis (Oval macrocytes) as well as other toxic nuclear effects (Howell-jolly ,hypersegmentation)

Lymphoblastic lymphoma

T vs B origin

morphology

WHO focuses on cytogenetic and molecular findings

acute lymphocytic leukemia

Differentiates ALL from othe acute nonlymphocytic leukemias and burkitt lymphoma

massive accumulation of primative lymphoid-appearing cells in a bone marrow occurs.

Characteristic of ALL; onset higest 1-5 years with a peak of  2-3 years of age; presents with lethargy, fever bone pain, with poor prognosis under 1 year of age.

A 50 year old woman who has been recieving busulfan for three years for chronic myelogenous leukemia becomes anemic. Laboratory test reveal:

Thrombocytopenia

Many peroxidase-negative blast cells in the peripheral blood

Bone marrow hypercellular in blast transformation

Markedly increased bone marrow TdT.

Which of the following complications is this patient most likely to have?

Acute lymphocytic leukemia

Recognize ALL as a possible progression for 1/3 of all CML cases.

increased resistance to infection

what IS?

pancytopenia, mononuclear cells with ruffled edges, splenomegaly as well as

decreased resistance to infection

Goucher cells

They DO inculde:flame cell, morula cells and grape cells.

Goucher cells are from a lipid storage disease.

Sheaths of immature plasma cells

differentiation of most common MM feature from others seen less frequently (flame) or diagnostic of other disease (Reed-Sternberg-Hodgkin lymphoma)

Wadenstrom macroglobulinemia

lymphoplasmic in origin

T lymphocytes

neoplastic line (sezery cells)

lysosomes

where accumulation of partially degraded mucopolysaccarides accumulate.

An oncology patient has the following results.

            DAY 1                             DAY 3

WBC:       8.0                                 2.0

RBC:        3.50                                3.45

Hgb:         10.0                                9.9

Hct:          28.8%                            29.5%   

Plt:            180                                150

The most propbble explanation is:  

A leukocyte and differential on a 40 year old Caucasian man revealed:

WBC:   5.4

Differential:

Segs:       20%

Lymphs:  58%

Monos:     20%

Eos:           2%

Thsi data represents:

A leukocyte count and differential on a 40 year old Caucasian man revealed:

WBC:  5.4

Differential:

Segs        20%

Lymphs: 58%

Monos:   20%

Eos:         2%

This represents:

Given the following data:

WBC: 8.5

Differential:

Segs       56%

Bands:     2%

Lymphs  30%

Monos:    6%

Eos:          6%

What is the absolute lymphocyte count?

2,550

8.5 X .30= 2,550

Given the following data:

 WBC: 8.5

differential:

Segs 5    6%

Bands:    2%

Lymphs: 30%

Monos   : 6%

Eos:        6%

What is the absolute eosinophil count?

510

0.06 X 8.5 = 510

(number of cells counted x dilution x10)

_______________________________

number of squares counted.

120 X 10 X 10

______________________________

8

12000

________

8

1,500

50 x100x10=50,000

50,000/8 =6,250

7500

Add 100 to the total number of nucleated red blood cells you observed per 100 WBCs. The total in this example is 300.

Use a calculator to divide the two totals. In this example, you divide  2250 by 300, so the corrected WBC count is approximately 7500. The corrected WBC count equals the uncorrected WBC count multiplied by 100, and this total divided by the number of nucleated red blood cells added to 100

8000

10,000 x100 =1000000/ 125=8,000

13,333

20.0 x 100 = 2000

50+ 100=150

2000/150=13,333

10.0

18X100=1800

1800/180 =10.0

A mean cellular hemoglobin concentration (MHCH) over 36g/dL is frquently found in:

Hereditary spherocytosis, lipemia (abnormally high plasma lipid)

active cold agglutinin disease

an elevate hematocrit

An elevated hematocrit decreases the amount of plasma in the whole blood and causes an effective increase in the amount of citrate added to that plasma. Recalcification incompletly overcomes the additional citrate, and prolongations in clotting time test can be expected, unless the amount of citrate is adjusted for the abnormal hematocrit.

Which of the following statements about this field is true?

[image]

nucleated cell in the lower right hand side if the image below (B)is a:

[image]

Myelocyte

this stage is where secondary granules first show up

The cells seen below are consistant with:

[image]

Cell description:

Size: 12 to 16 um

Nucleous: Oval, notched, folded over to horseshoe shape 

Chromatin: fine lacy

Nucleoli: none present

Cytoplasm: abundant, slate gray, with many fine lilac granules.

This cell is a

The large  in the image below is:

[image]

normal peroxidase activity

they are hypogranular

A 30 year old woman was admitted to the hospital for easy brusing and menorrhagia. Laboratory findings included the following:

WBC: 3.5

RBC: 2.48

Plt:     30

Hgb:   8.6

Hct:     25%

MCV:  100.7

MCH:   34.7

MCHC: 34.3

PT:     34.0 sec

APTT: 62.5 sec

TT: 15.0 sec

FSP: >40

fibrinogen: 315

Differential:

Polys: 3%

Lymphs: %

Monos: 2%

Abnormal immature : 58%

Blasts: 31%

nRBC: 1

Auer bodies, 1+ macrocytes, 1+ polychromasia

The cells identified as "abnormal immature" were described as having lobulated nuclei with prominent nucleoli; the cytoplasm had intense azurophilic granulation over the nucleus, with some cells containing 1-20 Auer bobies, frequently grouped in bundles, A 15-17 chromosome translocation was noted. Cells were SBB, peroxidase and NAS-D Chloroacetate positive, PAS negative, Which of the following types of acute leukemia is most likely?

CD19, CD20, CD21 and CD5

CLL lymphocytes express the pan-B cell markers as well as CD5, which is normally not expressed in normal B cells , but is on the surface of normal T-cells

In flow cytometric ananlysis, right angle (90 degrees) or side scatter of a laser

light beam provides information that pertains to a cells

CD138

CD138 or syndecan , is expressed on neoplastic plasma cells, and is useful in the diagnosis of myeloma

c-myc and the immunogloulin heavy chain

The (8;14) typically found in burkitt leukemia (ALL-L3) brings the master cell cycle control factor c-myc on chromosome 8 under the influence of the strong immunoglobulin heavy promoter on chromosome 14, driving a leukemogenic process

nuclear division without cytoplasmic division

nuclear maturation and diviosion occurs first, and is largly complete before cytoplasmic maturaton begins

5 days

5-8 days

The following results were obtained;

WBC: 1.8

Hgb:   8.9

Hct:  27.4

Plt:  2,300

LAP:  90

Differential

 Segs 70%

Bands 10%

Lymphs: 18%

Monos: 2%

Giant, Bizarre platelets, rare megakaryocytes

3+ Poikilocytosis, 2+ Anisocytosis, 1+ Schitocytes

This is consistant with:

A 53 year old man was in recovery following a triple by-pass operation. Oozing was noted from his surgical wound. The following laboratory data were obtained:

Hemoglobin 12.5

Hematocrit: 37%

Prothrombin time: 12.3

APTT: 34 seconds

Platelet count: 40

Fibrinogen: 250

The most likely cause of bleeding would be:

A 60 yerar old man has a painful right knee and a slightly enlarged spleen. Hematology results include

hemoglobin    15

absolute neutrophil count 10.0

platelet count 900,000

uncorrected retic count 1%

normal red cell morphology and indices

a slight increase in bands

rare metamyelocytes and myelocytes

giant and bizarre platelets

This is most compatible with

Cryoprcipitated AHF

DAVVP is better

366,000

183 x 200 x10/1

A phase platelet count was performed and the total platelet count was 356,000, 10 fields on the stained blood smear were examined for platelets and the results per field were 16,18,15,20,19,17,19,18,20,16

The next step would be to:

report the phase platelet count since it coorelated well with the slide.

Platelet count matches estimate well (average of 17.8 platelets per oil immersina field x 20,000)

In the platelet aggregation curve the

[image]

The aggregating agent was added and the curve began to clime and flattened out soon after the secondary wave. What is this typical of?

The following platelet aggregation tracing represnts :

no aggregation with ADP, collagen or epinephrine

normal with ristocetin

heparin inhibits the action of vitamin K

what is true:

There are two sources of vitamin  , one vegatable and one bacterial, Vitamine K converts precursor molucules into functional coagulation factors. Vitamine K is fat soluable

Fletcher factor deficiency (prekallikrein)

Prolonged APTT with a normal PT denotes a problem with the intrinsic pathway, so Factors VIII, IX, XI would be looked at, these deficiencies would correct in a mixing study, A characteristic of prekallikerin  deficiency is the correction of the PTT when incubated for 10 mins with KAOLIN, Celite,  silica, or egalic acid

The results on a patient are:

TEST                              PATIENT RESULTS    REF RANGE

PT                                                      18.5              11.0-13.5

APTT:                                                47.5              24-35

TT:                                                     14.0              12-19

ATIII                                                  82%             70-130%

Protein C                                            54%              77-167%

Protein S                                             48%              65-140%

activated protein C resistance             2.6               >2.1

These results reflect:                 

Warfarin

Warfarin is a Vitamine K antagonist

affecting factors II, VII IX and X

A four year old boy presents with chronic ear infections and is on prophylactic antibiotics. He presents with bleeding. Factors assays reveal:

TEST                       Patient results                 ref range

Factor VIII                  100%                         50-150%

Factor V                        75%                         50-150%

Factor IX                       38%                         50-150%

Factor II                         22%                         50-150

Possible caues   

The following laboratoy data were obtained from a 27 year old man with a long histiry of abnormal bleeding:

PT                                             normal

APTT                                        markedly prolonged

Factor VIII coagulant activity  markedly decreased

Factor VIII related antigen:      normal

Platelet count:                           normal

Template bleeding time            normal

Which of the following disorders does this man most likely have?

The following laboratory data were obtained fro a 40 year old woman with a long history of abnormal bleeding

PT;                                               normal

APTT:                                          prolonged

Factor VIII -coagulant activity   Decreased

Factor VIII related antigen          Markedly decreased

Platelet count                               normal

Template bleeding time              prolonged

Which of the following disorders does this woman most likely have 

APTT abnormal

Bleeding time  abnormal

Ristocetin cofactor abnormal

A patient presents with bleeding 48 hours post tooth extraction. Results are as follows:

TEST                     PATIENT RESULTS                 ref range

PT                                 11.5                                   10-13

APTT                            32.5                                   23-35

Fibrinogen                    345                                     200-400

Platelets                        324                                     150-400

Possible causes are a deficiency in:   

Type I

In a type I deficiency of protein S there is decreased activity assay. To determine if the deficiency is a dysfunctional molecule or a quantitative disorder, an ELISA assay should be performed to determine a type II deficiency 

FVL

A DNA test is available to confirmthe specific point mutation of activated protein C resistancenis FVL

Hypofibrinogenemia

A patient develops unexpected bleeding and the following test results were obtained:

PT and APTT                         prolonged

Fibrinogen                              Decreased

D-Dimer                                  increased

Platelets:                                 decreased

what is the most probable cause od these results:

Patient results are as follows:

TEST               Patient results                     reference range

PT                     17.5                                        11-13

APTT                56.7                                       25-35

D-dimer            698                                         <250

Fibrinogen        123                                         200-400

Platelet count    102                                        200-400

This workup suggest     

A patient develops severe unexpected bleeding following four transfusions. The following test results were obtained:

PT and APTT           prolonged

Platelets:                  50

fibrinogen:               30

DiDimer                   increased

given these results which of the following blood products should be recommended to the physician for this patient?  

monitors heparin therapy

intrinsic pathway

Protein C

Protein C is a vitamine K dependant protein , hence warfarin therapy will lower levels of this protein. 

DRVVT

If the initial APTT remains prolonged in a mix, a second assay should be performed. The lupus anticoagulant has multiple targets . The second test is the DRVVT, which, triggers coagulation at factor X

formed and are being excessively lysed

D-dimers are produced from crosslinked and stabilized fibrin clots.

Factor VIII activity

 Severe liver disease shows a decrease in hepatic synthesis of factors II, VII, IX and X< V, I and XI. The degree of decrease in each of these factors will be dependant on the degree  of fibrinolysis and the degree of elevatoion  of factors behaving as acute phase reactants, including  Factor VIII.

Activated clotting time

The ACT monitors high dose heparin therapy at clinics, bedside , cardiac catheterization or at the surgical suite. The stypvin time is a test using snake venom for testing for lupus. The reptilase time is used to distinguish between  heparin contamination  and a decreased fibrinogen level

make an appropriate dilution of the sample

+++ indicated that the wBC count exceeds the upper reporting limit.

review the stained blood smear

low platelet counts should be verified with a blood smear

Thr following results were obtained on an electronic particle counter:

WBC:       6.5

RBC:        4.55

Hgb:         18.0

Hct:          45.5

MCV:        90.1

MCH:        39.6

MCHC:      43.4

The first step in obtaining valid results id to: