hematology final Sara

Hematopoiesis

The process of making mature blood cells that come from the bone marrow (WBC, RBC, platelets)

Normal MCV range and how to calculate it

80-100 fl, hematocrit/RBC count=MCV

RDW

Red cell distribution width, increased RDW indicates the presence of cells of widely differing sizes

Microcytic iron deficiency

low MCV, high RDW

Microcytic thalassemia

Low MCV, normal RDW

Macrocytic

Very high MCV, B12 or folate deficiency

Normocytic

normal MCV

Microcytic anemia

MCV<80, severe iron deficiency and copper, reduced heme synthesis (lead poisoning, congenital or acquired sideroblastic anemia), reduced globin production

3 most common cause of microcytosis in clinical practices are?

Iron deficiency (low iron stores), alpha or beta thalassemia minor (normal or high iron stores), anemia of chronic disease (hepatoma, RCC)

Macrocytic anemia

MCV>100, reticulocytosis, abnormal nucleic acid metabolism of erythroid precursors (folate or B12 deficiency), abnormal RBC maturation (leukemia)

Common causes of macro anemia

Drugs interfering with nucleic acid synthesis, alcohol abuse, liver disease, hypothyroidism

Normocytic anemia

Normal MCV, increase distruction, reduced production (bone marrow suppression), acute blood loss, chronic renal failure.

Reduced production in normocytic anemia

Bone marrow invasion (myelofibrosis, multiple myeloma), myelodysplastic syndromes, aplastic anemia

Diagnosis of iron deficient anemia

Low serum iron, up TIBC, low transferrin saturation<20%, low ferritin <10

Iron deficient anemia

Iron replacement requires normalization of the hemoglobin and the body stores, always look for the cause.

Anemia of chronic disease

Iron cannot be remobilized from storage, blunted production of erythropoietin.

Lab findings of anemia of chronic disease

low TIBC, low iron, low transferrin, high ferritin, usually normocytic and normochromic but may be microcytic if severe.

Anemia of chronic disease is common in patients with?

Infection, cancer, inflammatory and rheumatologist diseases.

Sideroblastic anemia

Produces a dimorphic blood film with microcytes and macrocytes

Two common features of sideroblastic anemia

Ring sideroblast in the bone marrow, impaired heme biosynthesis.

Things associated with sideroblastic anemia/ causes

Myelodysplastic syndrome, drugs (ethanol, INH), toxins (lead, zinc), nutritional

Thalassemia

Anemia 2 degrees reduced or absent production of one or more globin chains.

thalassemia diagnosis

Poikilocytosis, hemoglobin electrophoresis is only diagnostic for beta thalassemia and may not be diagnostic for iron deficiency, Hb H prep or DNA analysis is needed to diagnose alpha thalassemia

Sickle cell disease

Inherited anemia (HBs)

Sickle cell treatment

Acute crisis includes fluids, oxygen, pain control, and transfusions, hydrocyurea, magnesium, clotrumazole, full vaccination is needed before hyposplenism develops, transplant

B12 deficiency

Megaloblastic anemia (macro, hypersegmented neutrophils, abnormal megaakaryocytes), be aware of neurological complications.

Treatment and testing for B12 deficiency

Never treat possible B12 deficiency with folate, the CNS lesions may progress, schilling test distinguishes pernicious anemia from other causes.

Folic acid deficiency

Women of child bearing age should take supplemental folate to prevent neural tube defects, folate supplementation lowers homocysteine levels leading to less heart disease and stroke.

Hemolytic anemia

Extravascular hemolysis, intramuscular hemolysis, immune (positive direct antiglobulin test), nonimmune (microangiopatjic hemolytic anemia)

Extravascular hemolysis

Increased reticulocytes, increased LDH, increased indirect bilirubin concentration, sickle cell

Intravascular hemolysis

RBC fragments, hemoglobinuria, urinary hemosiderin, decreased haptoglobin, G6PD

Approach to bruising and bleeding

Family history, drugs, other disease, check for spleen, Petechiae, purpura, and telangiectasia

Hemostasis investigations

Platelet count and platelet function, INR, PTT, fibrinogen, thrombin time, inhibitor studies.

Factor 7 VII

Made in liver, vitamin k dependent

Factor 5 V

Not vitamin k dependent

Factor 8 VIII

Made in endothelial cells

Platelets

Acquired disfunction is common in ill patients, blood cells help differentiate ITP from TTP, low platelet counts come from bone marrow

Approach to thrombocytopenia

Increase destruction (ITP, TTP, HIT, decreased production (amegakaryocytic, aplastic anemia, acute leukemia), other: drugs, connective tissue disease, HIV, hypersplenism

Thrombotic thrombocytopenic purpura diagnosis

Microangiopathic hemolytic anemia= non immune hemolysis with prominent red cell fragmentation.

Causes or symptoms of thrombotic thrombocytopenic purpura

Thrombocytopenia, acute renal insufficiency, neurological abnormalities, fever

Immune thrombocytopenic purpura

Acquired: post viral infections in children, immune idiopathic: mainstream of treatment is steroids, if steroids don’t work after two weeks consider splenectomy, in severe treat with IVIg (rapid response)

Hereditary disorders of secondary hemostasis

Hemophilia A, hemophilia B, DDAVP, von Willie brand

Hemophilia A

factor VIII deficiency

Hemophilia B

Factor IX deficiency, are X linked and produce hematomas and are treated with recombinant factor.

Von Willie brand

Prolonged bleeding time and prolonged PTT

Disorders of secondary hemostasis

Vitamin K deficiency, liver disease, circulating anticoagulant, DIC

Vitamin K deficiency factors

II, VII, IX, X

Liver disease consist of all factors except?

VIII

DIC

Uncontrolled thrombin and plasmin, excess thrombin leads to clotting and excess plasmin leads to bleeding, can be chronic

Disorders that trigger DIC

Sepsis, trauma, cancer, fat or amniotic fluid, acute promyelocytic leukemia

First 50

What does DIC produce?

RBC fragmentation, confusion or coma, renal failure, bleeding

Management of DIC

Treat the underlying cause, give cautious replacement therapy with FFP, cryoprecipitate and platelets.

Approach to neutropenia

History: drugs, toxins, recurring mouth sores, physical: splenomegaly, bone Pain, bone marrow

Slide plate of neutropenia

Granulocytic precursors or blasts present

Drug induced neutropenia

Antithyroid, antibiotics, sulpha, anticonvulsants, antipsychotics

Leukemoid reaction in CML

Mimicked by acute bacterial infection inflammatory reactions, severe marrow stress such as bleeding, underlying tumors

Leukemoid reaction in CLL

Mimicked by pertussis, TB, and mono

Leukemoid reaction in CMML

Mimicked by TB

Febrile transfusion reactions

Most common reaction within 1-6 hours of transfusion, most often due to cytokines in the product.

Transfusion reactions

Urticaria, anaphylaxis, acute hemolytic, delayed hemolytic, citrate toxicity

Urticaria

Soluble plasma substances react with donor IgE

Anaphylaxis

Sec-min, IgA deficiency

Acute hemolytic ABO mismatch

Recheck blood group and cross match

Delayed hemolytic

2-10 days, mimics AIHA

Citrate toxicity

Give calcium gluconate

Central causes of pancytopenia

Empty marrow, infiltration by abnormal cells, deranged marrow, starved marrow, drug induced

Peripheral causes of pancytopenia

Hypersplenism, autoimmune, severe sepsis

Aplastic anemia

Pancytopenia with empty marrow, most idiopathic cases are due to abnormal T cell inhibition of hematopoiesis.

Treatment for aplastic anemia

Immunosuppression, allogenic BMT

Myeloproliferative disorders

All disorders are pluripotent stem cell, acute myelogenous leukemia, acute lymphoblastic leukemia, chronic, myelodysplastic syndromes

Acute myelogenous leukemia

Usually in adults, blast are large with abundant cyptoplasm, auer rods and granules

Acute lymphoblastic leukemia

Usually on children, blast are small, complete remission and cure rate is high, some cases require bone marrow transplant

Myelodysplastic syndromes

Refectory anemia, refractory anemia with ring sideroblast, refractory anemia with excess blast, chronic myelomonocytic leukemia

Chronic myeloproliferative disorders

Polycythemia Rubra Vera, chronic myeloid leukemia, idiopathic myelofibrosis, essential thrombocythemia

Chronic lymphocytic leukemia

Common in elderly, lymphocytosis and smear cells, treatment is alkylating agents, radiation

Multiple myeloma

A monoclonal immunoglobulin in serum or single light chain is found in urine, renal failure and lytic bone lesions, Rouleaux

Macroglobulinemia

Monoclonal IgM protein, hyperviscosity with plasmapheresis

Hodgkin disease

The reed-sternburg cell is diagnostic, long term complications of heart disease, hypothyroid and malignancies

Thrombophilia

Antiphospholipid antibodies, factor V Leiden, deficiency of protein C, S, and Antithrombin

Production, development, and maturation of all cells

Hematopoiesis

Development order of red blood cells is?

Pronormoblasts, basophilic oblast, polychromatophilic normoblast, orthochromic normoblast, reticulocyte, erythrocyte

Accuracy

Describes how close the result is to the true value

Reference intervals

Values that have been established for a particular analyst method or instrument and particular patient population

Precision

Describes how close the test results are to one another when repeated analyses of the same material are performed

Which of these is a normal non critical hemoglobin value?

15 g/dL

Normal hemoglobin values

Males 14-18, females 12-16

Normal MCHC

32-36%

Normal MCH

27-29pg

Lab findings on a patient are MCV: 55fl, MCHC 25%, MCH:17pg

Microcytic hypochromic

What is the primary purpose of hemoglobin?

To deliver oxygen

Morphological classification of anemia is determined by?

Red cell indices

What test is used to monitor red cell production?

Reticulocyte count

I am adult the usual location for obtaining a bone marrow aspirate is the?

lilac crest

The majority of iron in an adult is found in?

Hemoglobin

Heinz bodies are

Unable to be seen with typical, commonly used staining methods

Following the rule of 3 which hemoglobin value would you suspect with a RBC value of 4?

12

Following rule of 3 what value would you expect with hematocrit levels at 12?

36

How to calculate MCH

Hgb/RBC X 10

How to calculate MCHC

Hgb/hematocrit X 100