Red cell/White cell disorders, Leukemia/Lymphoma (Peds) Flashcards
The physiologic nadir for hemoglobin in infants occurs at approximately?
2 months of age
What effect does anemia have on the hemoglobin-oxygen-dissociation curve?
This causes the affinity of hemoglobin for oxygen to become reduced in?
1) Concentration of 2,3-DPG increases within the RBC leading to the curve to shift to the right
2) Tissues needing to be oxygenated
At what rate of development for anemia does the body better compensate it?
What rate usually results in more dramatic symptoms?
1) Slowly developing anemia
2) Rapidly developing anemia
What does the MCV (mean corpuscular volume) tell you?
The mean volume of individual RBCs in a sample
What does the MCHC (mean corpuscular hemoglobin concentration) tell you?
Grams of HGB per 100 mL of RBCs
What does the RDW (Red blood cell distribution width) tell you?
Variability of RBC sizes in a sample
What does an H/H (hemoglobin/hematocrit) lab test tell you?
1) The amount of hemoglobin in the blood
2) The volume percentage of red blood cells in blood
What tells you if the RBCs are microcytic, normocytic, or macrocytic?
What tells you if the RBCs are hypochromic, normochromic, or hyperchromic?
1) MCV
2) MCHC
What are general causes of anemia?
1) Decreased RBC production (in bone marrow)
2) Increased RBC destruction
Hemoglobinuria, hemoglobinemia, and hemosiderinuria are due to what type of hemolysis?
Intravascular
Splenomegaly and hepatomegaly are due to what type of hemolysis?
Extravascular
Chronic blood loss will lead to what type of anemia?
Microcytic hypochromic anemia
A low number of reticulocytes in a patient with anemia is indicative of?
Inadequate bone marrow response
What is a normal bone marrow response to anemia?
Increased number of reticulocytes
What is a congenital pure red blood cell aplasia that almost always presents in infancy?
Diamond-Blackfan anemia
What does Diamond-Blackfan anemia result in?
Macrocytic anemia with low reticulocyte count
The most common inherited form of aplastic anemia is?
Fanconi anemia
What can fanconi anemia progress to?
We may not see symptoms until?
1) Pancytopenia
2) Around 10 years-old
Microcytic, hypochromic anemia in infants and toddlers is most often due to?
This is most likely caused by?
1) Iron deficiency
2) Dietary lack of iron
How could an peds patient present with iron deficiency anemia?
Pale and diet mostly made up of cow’s milk
Mentzer index is most helpful in distinguishing mild IDA from?
Beta thalassemia trait
If the Mentzer index is less than 13 it means?
If it’s greater than 13?
1) IDA
2) Beta thalassemia trait
How is absolute neutrophil count calculated?
ANC = ((%neutrophils + %bands) X (WBC)) / 100
Neutropenia occurs when a decrease in the absolute neutrophil count is less than?
Mild neutropenia: ANC = ?
Moderate neutropenia: ANC = ?
Severe neutropenia: ANC < ?
1) 1500/µL
2) 1000 - 1500
3) 500 – 1000
4) 500
What would lead poisoning show on a peripheral smear?
Basophilic stippling
If the ANC is < 500, the risk of what is high?
Serious infection
Impaired myeloid differentiation caused by maturational arrest of neutrophil precursors causes what condition?
Kostmann syndrome (Severe Congenital Neutropenia)
What are the clinical manifestations of Kostmann syndrome?
Life threatening pyogenic infections early in life
What is the inheritance of Kostmann syndrome?
Autosomal recessive
Kostmann syndrome increases the risk of?
AML
Cyclic fever, oral ulcers, gingivitis, periodontal disease, and recurrent bacterial infections are clinical manifestation of what condition?
Cyclic neutropenia
What is defected in cyclic neutropenia?
Stem cell regulatory defect
What is the inheritance of cyclic neutropenia?
Sporadic or autosomal dominant
What is the ANC for cyclic neutropenia?
ANC <200 for 3-7 days every 15-35 days
Shwachman-Diamond syndrome is due to a triad of what?
1) Neutropenia
2) Exocrine pancreatic insufficiency
3) Skeletal abnormalities
What is the inheritance of Shwachman-Diamond syndrome?
Autosomal recessive
Shwachman-Diamond syndrome increases the risk for?
Myelodysplastic syndrome or leukemia
What is defected in Shwachman-Diamond syndrome?
Neutrophil mobility
Bone marrow failure syndrome with all cell lines affected results in?
Fanconi anemia
What is the inheritance of Fanconi anemia?
Autosomal recessive
Fanconi anemia increases the risk for?
AML, brain tumors, and Wilms tumor
What does Leukocyte adhesion deficiency result in?
- Delayed separation of umbilical cord stump (>3 weeks)
- Recurrent and severe bacterial and fungal infections without pus accumulation
- Poor wound healing
What is the inheritance of Leukocyte adhesion deficiency?
Autosomal recessive
What is the cause of Leukocyte adhesion deficiency?
Neutrophils have diminished adhesion to surfaces so they can’t migrate out of blood vessels
Hyperimmunoglobulin E syndrome (Job syndrome) causes a triad of?
- Severe eczema
- Recurrent bacterial infections (usually staph) of skin
- Recurrent pulmonary infections (bacterial, fungal)
What is the inheritance of Hyperimmunoglobulin E syndrome (Job syndrome)?
Sporadic or autosomal dominant
What is a very rare autosomal recessive disease that causes partial oculocutaneous albinism, neuropathies, and recurrent pyogenic infections?
Chediak-Higashi syndrome
Chronic granulomatous disease is due to recurrent purulent infections with?
Fungal or bacterial catalase-positive organisms
Chronic granulomatous disease is due to absent?
Superoxide
What is the inheritance of Chronic granulomatous disease?
X-linked recessive
What virus causes neutropenia and causes a slapped cheek appearance?
Parvovirus B19
DiGeorge syndrome is associated with an absent?
This results in what type of immunodeficiency?
1) Thymus
2) T-cell immunodeficiency
The #1 cause of death of children due to illness is?
Brain Cancer
The most common malignancy between age 15-19 years-of-age is?
Hodgkin Lymphoma (HL)
What infectious agent may be involved with Hodgkin lymphoma?
EBV
What are considered a pathognomonic feature of HL?
Reed-Sternberg cells
What are the B-signs used in the staging of Hodgkin lymphoma?
1) Unexplained fever
2) Weight loss
3) Night sweats
Any patient with persistent, unexplained LAD unassociated with an obvious underlying inflammatory or infectious process should undergo?
Especially if?
1) CXR to rule out a mediastinal mass
2) They display respiratory symptoms
What is the inheritance of Wiskott-Aldrich syndrome?
X-linked recessive
What is the classic triad of Wiskott-Aldrich syndrome?
1) Recurrent sinopulmonary and ear infections
2) Severe atopic dermatitis
3) Bleeding secondary to significant thrombocytopenia
Wiskott-Aldrich syndrome predisposes a patient to?
Non-Hodgkin lymphoma
What condition commonly manifests as abdominal (sporadic type) or head and neck disease (endemic type) with involvement of the bone marrow or CNS?
Burkitt Lymphoma
What account for the greatest percentage childhood malignancies?
Leukemias
Trisomy 21 (Down Syndrome) is associated with what leukemias?
ALL and AML
At what platelet count is clinically significant bleeding possible?
When is life threatening hemorrhage possible?
1) Less than 20,000 μl
2) Less than 10,000 μl
The decreased platelet counts seen in Immune thrombocytopenic purpura is due to?
The platelets that are present have what distinct feature?
What precipitates ITP by about 1-2 weeks?
What do you treat it with?
1) Anti-platelet antibodies
2) Large
3) Viral illness
4) Prednisone
What is Kasabach-Merritt phenomenon due to?
What is it associated with?
1) Thrombocytopenia and hypofibrinogenemia
2) Giant hemangioma and intravascular coagulation
What are the signs and symptoms that are “red flags” in patients with thrombocytopenia?
1) Pancytopenia
2) Raised LDH (elevated in cancer)
3) Associated new renal impairment
Hemolytic Uremic syndrome presents as classic triad of?
1) Microangiopathic hemolytic anemia
2) Thrombocytopenia
3) Renal damage
