All Lecture Notes Flashcards by Leslie Taber

What do RBCs contain that are essential for oxygen transport?

hemoglobin

RBCs are responsible for transporting oxygen throughout the body.

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List the requirements for RBC production.

Folic acid
Iron
Erythropoietin (made by kidneys)
Vitamin B12

These nutrients are crucial for the production of red blood cells.

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What does CBC stand for and what is its significance?

Complete Blood Count

It is the most common and valuable tool to evaluate hematologic function/dysfunction.

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What is the normal RBC count range?

4.5-5.5

A low count indicates insufficient production by the marrow, while a high count suggests increased production in response to a condition.

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What are the normal ranges for Hgb and Hct?

Hgb: 11.5-15.5
Hct: 35%-45%

These values are critical for assessing oxygen-carrying capacity and blood volume.

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What does a reticulocyte count indicate?

Bone marrow function

A normal reticulocyte count is 0.5%-1.5% of erythrocytes.

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What are the three kinds of anemia based on RBC production?

Decreased RBC production
Increased RBC loss
Increased RBC destruction

Each type has distinct causes and clinical manifestations.

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What are the symptoms of decreased RBC production?

Pallor
Tachycardia
Headache
Fatigue
Shortness of breath
Muscle weakness
Systolic heart murmur
Pica

These symptoms arise from insufficient oxygen delivery to tissues.

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What causes iron deficiency anemia?

Decreased intake
Excessive use
Chronic disease
Chronic blood loss

It results in smaller RBCs and decreased hemoglobin concentration.

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What is aplastic anemia?

Total bone marrow failure

It results in pancytopenia, where all cell lines are depressed.

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What are the clinical manifestations of increased RBC loss?

Pallor
Fatigue
Headache
Muscle weakness
Cool skin
Tachycardia
Decreased peripheral pulses
Low blood pressure

These signs indicate a significant drop in blood volume.

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What is hemolytic anemia/sickle cell?

Normal Hgb (Hgb A) replaced with abnormal sickle-shaped Hgb (Hgb S)

This condition leads to various complications due to sickling of RBCs.

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What are the clinical manifestations of sickle cell anemia (SCA)?

Painful swelling of hands and feet
Abdominal pain
Strokes
Visual disturbances
Acute chest syndrome
Obstructive jaundice
Hematuria

These symptoms result from vascular obstruction and tissue ischemia.

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What is the therapeutic management for SCA?

Prevent sickling
Minimize energy expenditure
Hydration
Electrolyte replacement
Analgesia for severe pain
Blood transfusion/exchange transfusion
Antibiotic therapy
Hematopoietic stem cell transplant

These interventions aim to manage symptoms and prevent complications.

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What does SCID stand for?

Severe Combined Immune Deficiency

It is characterized by the absence of both humoral and cell-mediated immunity.

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What are the clinical manifestations of SCID?

Chronic infections
Failure to recover from infections
Frequent reinfection
Infection with unusual agents
Failure to thrive

These symptoms indicate a severely compromised immune system.

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What are platelets and their role?

Cellular fragments needed for blood clotting

Clotting also requires many coagulation proteins/factors.

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What is the normal platelet count range?

150-400 x 10^3/mm^3

This range is essential for proper clotting function.

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What are the three steps of complex clotting?

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Release of clotting factors from injury site
Platelet plug formation
Thrombin formation

These steps are crucial for hemostasis.

What is hemophilia?

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Absence, deficiency, or dysfunction of coagulation proteins or factors

There are three types: Hemophilia A, Hemophilia B, and Von Willebrand Disease.

What is DIC?

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Disseminated Intravascular Coagulation

It is a secondary disorder characterized by widespread clotting and bleeding.

What are the clinical manifestations of DIC?

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Bleeding and clotting simultaneously
Petechiae
Purpura
Bleeding from skin openings
Hypotension
Organ dysfunction

These signs indicate severe coagulopathy and tissue ischemia.

What is beta thalassemia?

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A genetic blood disorder causing insufficient beta-globin production

It leads to fragile RBCs and severe anemia.

How is beta thalassemia inherited?

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Autosomal recessive pattern

Both parents must pass the faulty gene for the severe form (thalassemia major) to occur.

What are the **diagnostic indicators** for beta thalassemia?

* Small, pale red blood cells * Low hemoglobin and hematocrit * Immature red cells ## Footnote Hemoglobin electrophoresis confirms the diagnosis.

What happens to **red blood cells** due to unstable hemoglobin molecules?

They die early ## Footnote The body attempts to produce more red blood cells, but they remain defective.

What condition is caused by **iron buildup** in organs due to frequent transfusions?

Hemosiderosis ## Footnote This buildup occurs in the skin, liver, and lungs, and there is no natural way to remove excess iron.

What are the **early signs** of the condition described?

* Pale skin * Poor feeding * Fever * Enlarged spleen or liver ## Footnote These symptoms usually appear in infancy or toddlerhood.

What are some **ongoing symptoms** of the condition?

* Fatigue * Bone pain * Headaches * Shortness of breath * Poor growth * Delayed puberty ## Footnote Physical changes may occur if untreated.

What are the **main goals** of treatment and management?

* Keep hemoglobin levels high enough to support growth * Prevent iron overload ## Footnote Regular blood transfusions and iron chelation therapy are key treatments.

What is the recommended frequency for **regular blood transfusions**?

Every 2–5 weeks ## Footnote The goal is to maintain hemoglobin around 9.5 g/dL.

Name the **medications** used in iron chelation therapy.

* Deferoxamine * Deferasirox * Deferiprone ## Footnote These medications help remove excess iron from the body.

What is a potential **surgical intervention** for the condition?

Splenectomy ## Footnote This may be needed if the spleen destroys too many red cells.

What are the **risks** associated with the condition?

* Heart and liver damage from iron overload * Infections after splenectomy ## Footnote Stem cell transplant can cure some children, especially if done early.

What is the **prognosis** with early and consistent treatment?

Children can live into adulthood with good quality of life ## Footnote Risks include heart and liver damage from iron overload.

What is **Immune Thrombocytopenia (ITP)**?

An acquired bleeding disorder where the immune system destroys platelets ## Footnote Most common in children under 10, often following a viral illness.

What is the **main problem** in ITP?

Low platelet count due to immune destruction ## Footnote This leads to easy bruising and bleeding.

What are the **visible signs** of ITP?

* Easy bruising * Petechiae * Ecchymoses * Mucosal bleeding ## Footnote Internal bleeding signs include blood in urine and vomiting blood.

What is the **therapeutic management** for ITP?

* Supportive care * Medications like Prednisone and IVIG * Splenectomy for chronic cases ## Footnote Most cases resolve on their own.

What is the **prognosis** for children with ITP?

Most recover without complications ## Footnote Some may develop chronic ITP and require further treatment.

What are **Immunologic Deficiency Disorders**?

Conditions where the immune system is too weak or misguided ## Footnote Examples include HIV/AIDS, SCID, and autoimmune disorders.

What is the **main problem** with immunologic deficiency disorders?

The body can’t mount a proper immune response ## Footnote This leads to frequent, severe infections and poor recovery.

What are the **diagnostic tests** for HIV/AIDS?

* ELISA & Western blot for children >18 months * PCR testing for infants ## Footnote Rapid tests are available for quick screening.

What are the **clinical manifestations** of HIV?

* Swollen lymph nodes * Enlarged liver/spleen * Oral thrush * Chronic diarrhea ## Footnote AIDS-defining illnesses include PCP pneumonia and recurrent infections.

What is the **therapeutic management** for SCID?

* Stem cell transplant * IVIG for passive immunity * Gene therapy is emerging ## Footnote A sterile environment is crucial to prevent infections.

What is the **prognosis** for SCID with early transplant?

Excellent prognosis ## Footnote Poor outcome without a donor.

What nursing care management is essential for HIV?

* Education on transmission * Support for families * Monitor growth and nutrition ## Footnote School safety and nutritional support are also important.

All Lecture Notes Flashcards

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