Hematology Comp exam review

_______ is done to confirm the diagnosis of Beta Thalassemia

Cryoprecipitate is obtained by centrifuging plasma and removing the precipitate. It is a blood product used to replace low

Beta Thalassemia is an intrinsic or ______; it is also autosomal ____

Acute episodes of ITP may arise in children following a _______ or another trigger, such as _____

ITP Labs include plt < _____, _____ PT, PTT, fibrinogen & Fibrin; Will have _____ on smear

Expected Von Willebrand disease lab levels:
PT: ____
PTT: ____
BT: ____

First-line treatment for an acute bleed event in hemophilia is _____

Initial workup for hemophilia that suspicious for msk bleeding includes a _____ and testing for _____ levels

Duration of abx tx for Acute chest is generally ______

_____ from decreased tissue perfusion from clotting is commonly seen on physical examination in patients with DIC

The best initial treatment of disseminated intravascular coagulation (DIC) is....

Patients with DIC who are not actively bleeding should receive prophylactic ______ with _____ or _____

Due to the immature liver in the newborn, ______ clotting factors are ______ for the first few months of life.

The normal neonatal hemoglobin level is _____

Hemophilia Labs
PT: ____
PTT: ____
Factor assay: ____

HUS often follows an episode of _______, and caused by infection with _______ -producing bacteria such as ______

Splenic sequestration crisis seen in SCD presents as _____ and ______ tenderness.

Peripheral blood smear showing ______, ____ RBCs with ______ cells is the typical picture of a patient with thalassemia.

Hemophilia is an ________ bleeding disorder caused by deficient or defective factor ______ or factor _______ & is characterized by _______

DDAVP stimulates the release of _____ & _____; Used to tx ______ & _____

Goals of exercise in pt with hemophilia include building ______, _____ and _____ endurance)

Mild Hemophilia is classified as ____% normal factor activity and may have _____

Moderate Hemophilia is classified as ____% normal factor activity and may have _____

Severe Hemophilia is classified as ____% normal factor activity and may have _____ and usually presents w/in _____

Hemophilia A is deficient in factor ____

Hemophilia B is deficient in factor ___

Von Willebrand’s protein functions to facilitate ___

Von Willebrand disease is an autosomal _______ bleeding disorder caused by decreased ______ or _______ of the pro-von Willebrand factor

Pts w/ Von Willebrand disease may complain of ________, _________, and _________

Aminocaproic acid (Amicar) is an antifibrinolytic used in the treatment of ______ for ______

Medications that cause an increased risk of hypercoagulability/ Thromboembolytic Disorders include _____, ______, & _______

Inherited risk factors for hypercoagulability/ Thromboembolytic Disorders include _____, _____, _____, _____, & _______

Goals of Hypercoagulability/ Thromboembolytic Disorders treatment include ________, _______, ______, & ________

Post-thrombotic syndrome is chronic _________ w/ ______, _____, & ______

Thalasemia

Beta Thalassemia Major is the

Beta Thalassemia major: Severe hemolytic anemia develops by ____ to _____ months of age

Sickle cell disease is a group of hemoglobinopathies caused by

Common triggers for a Vaso occlusive crisis are _______, _______, _______, ________

Hereditary Spherocytosis: _______ defect in the RBC causing _________

G6PD is an essential enzyme in ________

Hemolytic Uremic Syndrome (HUS) occurs when there is

The anemia in HUS is

Hemolytic Uremic Syndrome (HUS) is one of the main causes of ________ in children

Intrinsic hemolytic anemias are inherited or congenital abnormalities of _______, _______, or _________

Extrinsic hemolytic anemia are acquired from forces or agents that __________

Immune thrombocytopenic purpura (ITP) is an autoimmune disease characterized by ______ , ________, and ______ episodes caused by ________. Precipitated by _____. peak age = ______

Henoch-Schonlein Purpura (HSP) is an acute, systemic, _______ mediated __________ with inflammation/hemorrhage in the ____, _____, _____, & _____ System

Henoch-Schonlein Purpura (HSP) is primarily seen in _____. Peak incidence in ______ & is proceeded by ______

Heparin Induced Thrombocytopenia (HIT) is an immune mediated ______ reaction against endogenous _______ causing catastrophic ________. Theres also Increased ________ that r/in ________

ineffective hematopoiesis is....

Macrocytic anemias will have an MCV > ____

Microcytic anemias will have an MCV < ____

Normocytic anemias will have an MCV of _____ to _______. Generally things that r/t _______ or _____

What is Aplastic Anemia?

Aplastic anemia presents as anemia s/s + _______, _______, and _____

Aplastic anemia labs will have a ___Hbg, ___ WBC, ___ platelet; ___ or ____ retic count; ______ on smear; ___ or ____ hematopoietic elements from BMA

What is Transient Erythroblastopenia of Childhood (TEC), and what ages is it seen in?

Most Pts w/ Transient Erythroblastopenia of Childhood (TEC) present with

What is Diamond-Blackfan Anemia, and what is the etiology?

Children with Diamond–Blackfan anemia have an increased percentage of

What is the Management of Diamond-Blackfan Anemia?

Blood represents __% of the total body weight. It is made up of __ % plasma and __% of formed elements

Extrinsic clotting pathway begins with _______ that leads to the release of ________, which then converts _______ to _____

The intrinsic clotting pathway occurs when there's ________

Activated factor X converts ______ to ______

Thrombin helps us convert ______ to _______

Hematopoiesis is the process of

Anemia is the _________ and _______

Hemolysis is....

Immune Thrombocytopenic Purpura (ITP) peak incidence between ___ to ___ years old, often comes after a _____

Immune Thrombocytopenic Purpura (ITP) is an autoimmune disorder characterized by _______ leading to their _______

ITP acute tx: _______ for _____; ______ or ______

Methemoglobinemia is an uncommon cause of cyanosis in infants and children that is a result of methemoglobin containing

Acquired methemoglobinemia is generally caused from exposure to certain medications or agents that cause an increase in the production of methemoglobin which includes

Methemoglobinemia will have _______

__________ = confirmation of methemoglobinemia & level of methemoglobin.

Severe s/s of methemoglobinemia is managed with _______ if this fails it could mean_______

Hemophilia: Uncontrollable bleeding after injury 30-50% earliest symptom is _______

Factor administration for hemophilia pts: major bleed: ______ replacement minor bleed: ____ to _____ mucocutaneous bleed: ____ to ____

Factor VIII halflife= _____ to _____; Factor iX halflife =____ to ____

Pt/family education for hemophilia pts: Avoid _____ & _____ bc they ______. Vaccine for ___ & ____ recommended. and they need follow up how often

Hemophillia is an ______ disorder that is most common in ____. Hemophillia A is deficient in ____ & B is deficient in ____

_____ for mild-mod hemophilia A

G6PD is an enzyme that is crucial in ______

Glucose-6-Phosphate Dehydrogenase Deficiency is an ______ disease that is more common in _____

Children with G6PD-deficient RBCs are susceptible to _____ and _______ during certain conditions of stress, exposure to certain medications, foods, or chemicals.

G6PD-deficiency: Acute exacerbations may occur with the ingestion of _____, during the course of an infection, and during exposure to ______

Non-Hodgkin Lymphoma (NHL) is more common in ____ to ____. Hodgking lymphoma more common in _____

Hodgkin Lymphoma spreads to ______ & has _____ secretion

Lymphadenopathy in Hodgkins vs. Non-Hodgkins lymphoma

Vonwillibrand disease is _______ d/o with ______ but ______. Tx of choice = _____

A bone marrow aspirate with ______ is diagnostic for leaukemia

The induction phase of Leukemia tx/management is the ______; reduce resistance w/ ______, _______, & _______

The consolidation phase of Leukemia tx/management is the ______ with ______, ______, _____, _____ & ______

The maintenance phase (2-3 yrs) of Leukemia tx/management is to maintain remission with ________, ______, & ______

Complications of leukemia include

HLH: Aggressive, life-threatening syndrome of _________. (Cytokine storm--> immune cell damage and inflammation--> end organ damage)

Primary HLH – seen in _____ & _____. Often ______ or _______ that leads to HLH.

Secondary HLH – seen in ______, triggered secondary to medical condition with an underlying immune system defect. trigger can be malignancy, immune deficiency, specific infections such as _____ or _____

HLH Diagnosis 5 out of 8 criteria:

HLH tx

Macrophage Activation Syndrome (MAS) is HLH in setting of

Macrophage Activation Syndrome (MAS) is a severe complication of

Macrophage Activation Syndrome (MAS) tx:

Nutritional causes of iron-deficiency anemia result in ______. Excessive milk ingestion is associated with anemia, as milk....

ANC is used to determine ________. A normal ANC is ______. An ANC of _____ is of concern

The ANC is calculated by multiplying the WBC count times the percent of neutrophils (segmented + bands) in the WBC differential count. In order to calculate this: WBC = 300; % of polys = 10/100, or 0.10; % of bands = 5/100, or 0.05. Example: 300 × (0.1 + 0.05) = 45. The normal range for ANC is between 1,500/mm3 and 8,000/mm3. Reminder: neutrophils = polys + bands.