Hematologic and Immunologic Dysfunction Flashcards

Vocabulary flashcards covering pediatric hematologic and immunologic disorders, including diagnostic labs, anemia, sickle cell disease, and immune deficiencies.

Hemoglobin F

The predominant type of hemoglobin for the first $6\text{-months}$ of life.

Absolute Neutrophil Count (ANC)

A calculation used to determine infection risk, defined as (\text{% of Segs} + \text{% of Bands}) \times \text{Total WBC}.

Left Shift

An increase in the percentage of bands (immature neutrophils) in a CBC, which is classic for a bacterial infection.

Platelet count < 50,000/\bmu\text{L}

The threshold at which extreme caution is required due to the risk of spontaneous intracranial hemorrhage, necessitating quiet activities.

Neutropenia Emergency

In an immunocompromised child, a temperature reading of $> 100.4^{\text{o}}\text{F}$ ($38^{\text{o}}\text{C}$) is considered a medical emergency requiring immediate evaluation.

Hemodilution

A circulatory effect of anemia where the blood becomes thinner, potentially leading to increased cardiac circulation, turbulence, and murmurs.

Iron Deficiency Anemia (IDA)

The most prevalent nutritional disorder in the US, occurring when the body lacks sufficient iron to produce hemoglobin ($Hgb$).

Oral Iron Administration

A procedure where iron is best absorbed in an acidic environment and should be given through a straw or back of the mouth to prevent the adverse effect of staining teeth.

Pancytopenia

The simultaneous depression of all formed elements of the blood (RBCs, WBCs, and platelets), characteristic of Aplastic Anemia.

Hemoglobin S ($HbS$)

An abnormal hemoglobin structure found in Sickle Cell Anemia, caused by a mutation where the sixth amino acid in the $\beta\text{-globin}$ gene changes from glutamic acid to valine.

Vaso-occlusive (VOC) thrombotic crisis

The most common type of sickle cell crisis, characterized by blood stasis and clumping in microcirculation, leading to ischemia, infarction, fever, and severe pain.

Splenic sequestration

A life-threatening sickle cell crisis where large volumes of blood pool in the spleen, causing profound anemia, hypovolemia, and shock.

Acute Chest Syndrome (ACS)

A serious complication of sickle cell disease defined by a new pulmonary infiltrate, chest pain, fever, cough, tachypnea, wheezing, and hypoxia.

Hydroxyurea

A palliative treatment for sickle cell anemia that increases the production of Fetal Hemoglobin ($Hgb F$) to reduce sickling episodes.

Hemosiderosis

Iron overload in tissues, often manifesting as a bronze skin tone, caused by repeated blood transfusions in patients with $\beta\text{-Thalassemia}$.

Iron-chelating drugs

Medications such as deferoxamine or deferasirox used to bind excess iron for excretion via the kidneys in patients with iron overload.

Idiopathic Thrombocytopenic Purpura (ITP)

An acquired autoimmune hemorrhagic disorder characterized by the destruction of platelets in the spleen, often presenting with petechiae and a platelet count less than 20,000/\bmu\text{L}.

Hemophilia A

A classic hereditary bleeding disorder caused by a deficiency of clotting factor $VIII$ ($81$).

Hemarthrosis

Bleeding into joint spaces (knee, ankle, elbow), which is a common manifestation of hemophilia causing stiffness, warmth, redness, and severe pain.

RICE protocol

A nursing intervention for controlling bleeding in hemophilia consisting of Rest (Immobilization), Ice, Compression, and Elevation.

Severe Combined Immunodeficiency Disease (SCID)

A defect characterized by the absence of both humoral and cell-mediated immunity, requiring a sterile environment and bone marrow transplant.

Pneumocystis carinii pneumonia (PCP)

The most common opportunistic infection in children with HIV, typically occurring between $3$ and $6\text{-months}$ of age.

Vertical transmission

The primary route of HIV infection in children (93% of cases), occurring via prenatal or perinatal exposure or through breast milk from an infected mother.