Chapter 24- Hematologic function in children

Question 1

sickle cell

Answer 1

autosomal recessive, production of hemoglobin S within erythrocytes
under decreased O2 and dehydration, Hb s stretches looking like sickle cell; cells die prematurely= hemolytic anemia
sickled cells plug blood vessels= vascular occlusion, pain, infarction
at least 6 months old

Question 2

sickle cell s/s

Answer 2

mild to repeated vasooclussive crisis
hemolytic anemia= pallor, fatigue, jaundice, irritability

Question 3

thrombocytopenia purupra in children

Answer 3

most common disorder of platelet consumption
autoantibodies cause platelet sequestration & destruction of mononuclear phagocytes in spleen/lymphoid tissue that exceed bone marrow ability to produce
70% d/t viral illness (EBV, parvovirus, URI)

Question 4

s/s thrombocytopenia purupra in children

Answer 4

THINK bleeding
bruising
generalized petechial rash
ecchymoses from petechiae
asymmetric bruising legs/trunk
hemorrhagic bullae of gums/lips/mm
Appears well otherwise

Question 5

hodgkin’s lymphoma in children

Answer 5

proliferation of malignant lymphocytes in lymphoid system
RS cells from B cells
spreads contiguous way to lymphoid tissue
rare before age 5
boy > girls

Question 6

s/s iron deficiency anemia in children

Answer 6

highest 6 months- 2 years, adolescent girls, women child bearing age
nonspecific= irritability, decreased activity tolerance, weakness, lack of interest in play
pallor
anorexia
tachycardia
systolic murmurs
splenomegaly
widened skull sutures
decreased growth
developmental delays
pica

Question 7

Acute crises in sickle cell

Answer 7

1. vasooclusive crisis (extremely painful)
2. sequestration crisis (< 5 y/o, blood pool in liver/spleen)
3. aplastic crisis (bone marrow doesn’t compensate for lost erythrocytes d/t premature hemolysis)
4. hyperhemolytic crisis

Question 8

alpha and beta thalassemias

Answer 8

autosomal recessive disorders= impaired synthesis of one of two chain (alpha/beta) of adult hemoglobin
beta= more common
anemia, tissue hypoxia, red cell hemolysis

Question 9

s/s thalassemia

Answer 9

mild splenomegaly
bronze coloring of skin
hyperplasia of bone marrow
impaired growth/development
anemia > high output CHF
liver enlargement
limb-length discrepancy
chipmunk face

Question 10

s/s Hodgkin’s lymphoma in child

Answer 10

*painless lymphadenopathy in lower cervical chain
*with or w/o fever
anorexia, malaise, fatigue
weight loss
intermittent fever
night sweats
mediastinal involvement could put pressure on trachea= airway obstruction
extranodal primary sites rare