Anemia Flashcards
Physiologic Anemia
• The most common cause of anemia in young infants. • Between six to nine weeks of age. • It is due to decreased erythropoiesis (Due to improved tissues oxygenation in comparison with fetal life) and shorter RBCs life span.
Risk Factors
Of IDA
• Exclusively breast-fed infants who
continue on exclusive breast feeding
after 6 months of life. • Prematurity. • Infants fed cow’s milk when younger
than 1 year of age. • Toddlers fed large volumes of cow’s
milk. • Menstruating adolescent females who
are not receiving supplemental iron.
Cl inical Associations of IDA
In addition to anemia symptoms: • Poor muscle strength, gastrointestinal dysfunction, and impaired WBC and T- cell function • It is associated with later cognitive deficits and poor school performance.
Diagnosis of IDA
• Microcytic, hypochromic anemia (low
MCV and MCH). • High RDW. • Low serum ferritin. • If the history is consistent with IDA,
CBC usually sufficient without the need
for the iron profile.
Management of IDA
Dietary advice (Decrease cow’s milk intake, increase intake of iron- containing items) • Elemental iron for at least 3 months with follow up blood test. • The usual therapeutic dose of elemental iron induces an increase in hemoglobin of 0.25-0.4 g/dL per day • If the Hemoglobin failed to increase, careful re-evaluation for ongoing blood loss, development of infection, poor compliance, malabsorption, or other causes of microcytic anemia is required.
Causes of Red Cell Aplasia
• Congenital red cell aplasia (Diamond–
Blackfan anemia). • Transient erythroblastopenia of
childhood. • Parvovirus B19 infection (in children
with inherited hemolytic anemias).
Clues for Red Cells Aplasia
• Low reticulocyte count despite low Hb • Normal bilirubin • Negative direct antiglobulin test
(Coombs test) • Absent red cell precursors on bone
marrow examination.
Diamond– Blackfan anemia
• Genetic disease (Autosomal Dominant). • 80% sporadic. • Presents with anemia between 2-3 months of age. • Associated with short stature, abnormal thumbs. • Treated with steroid but if does not respond may require regular Blood transfusions. • BMT can cause cure.
Fanconi Anemia
This is the most common inherited form of aplastic anemia (Bone Marrow Failure). • Autosomal Recessive. • Associated with congenital abnormalities: short stature, abnormal radii and thumbs, renal malformations, microphthalmia, and pigmented skin lesions. • Hematological manifestations appear after 5 years of age. (pancytopenia) • Increased risk of malignancies due to DNA repair issues.
Autoimmune hemolytic anemia
• Acute, self limiting condition.
• Caused by production of Antibodies that
cause RBCs production.
• Can follow certain infections (EBV,
Mycoplasm, URTI) or associated with other
autoimmune diseases or drugs (penicillin). • Presents with symptoms of anemia.
• Investigations: Coombs positive, incresed
reticulocyte count and indirect biliriubin. • 80% resolve spontaneously.
• Treatment: Transfusion not always
effective (Why?), steroid in severe cases
only.
SCD
ماعندهم بيتا فماعندهم HB A
Sickle Cell Anemia
Dactylitis can be the presenting sign in
infants.
Sickle Cell Anemia CP
Si ckle Cell Anemia
Dactylitis can be the presenting sign in
infants.
Main clinical features are: - Anemia, - Infection, - Painful vaso-occlusive crises, - Sequestration crises, - Splenomegaly in some young children, - Growth failure, - Behavior and learning problems.
Types of crises: SCA
1- Vaso-occlusive crisis: - Intermittent episodes of acute pain. 2- Splenic Sequestration crisis: - Sudden splenomegaly and pallor, BP and Hb drop. 3- Hyperhemolytic crisis:
- Sudden increase in hemolysis with worsening anemia. 4- Aplastic crisis. (Caused by Parvovirus B19).
The most serious clinical complications SCA
The most serious clinical complications
are bacterial infection, acute chest
syndrome, strokes and priapism
(persistent, usually painful erection).
Management of SCA
• Prophylactic penicillin. • Immunization against encapsulated
bacteria. • Folic acid (Why?). • Maintain good hydration. • Treat crises. • Long-term: hydroxyurea or occasionally
bone marrow transplant
B -Thalassaemia major
Splenomegaly and hepatomegaly
Hemostasis
• Prothrombin time (PT) – measures the
activity of factors 2, 5, 7 and 10. • Activated partial thromboplastin time
(APTT) – measures the activity of
factors 2, 5, 8, 9, 10, 11 and 12.
History of bleeding
Age of onset • Site of bleeding: skin (petechiae,
bruising) mucus membrane (epistaxis,
GIT), deep tissue (joints, muscles) • Always check for other bleeding site • Skin and mucus membrane bleeding
may suggest platelets disorders or in
their interaction with blood vessels. • Soft tissue, muscle, and joints bleeding
suggest coagulation factors’ disorders.
• Did the symptoms correlate with the degree of injury or trauma? • In Acute cases: - History of recent URTI - History of constitutional symptoms (fever, weight loss, decrease appetite) • Child abuse should be considered in unexplained recurrent bleeding.
Family history: Bleeding history only in male siblings and maternal uncles is suggestive of X-linked recessive diseases, such as hemophilia A or B. Drug history (herbal medicine, warfarin poisoning, NSAIDs).
Diagnostic Testing in bleeding
Diagnostic Testing
• CBC including platelet count. • Peripheral Blood smear. • Prothrombin time (PT). • Activated partial thromboplastin time
(APTT). • Other lab workups depend on the case
history.
Hemophilia
• Hemophilia A – Factor 8 deficiency. • Hemophilia B- Factor 9 deficiency. • X-Linked recessive. • Hemophilia A is more common.
Bleeding Sites in hemophilia
• Hemarthrosis (ie, hemorrhage into a joint) is the most common site for bleeding in ambulatory patients, representing up to 80 % of hemorrhages. • Spontaneous Hemoarthrosis is characteristic of severe disease.
Bleeding into muscles with hematoma
formation is common. • In severe cases may cause
compartment syndrome.
Hematuria is a frequent manifestation
of severe hemophilia.
Ma nagement of hemophilia
Ma nagement • Replacement therapy for acute bleeding is the hallmark of hemophilia management. • • The desired level of factor’s activity depends on the bleeding severity.
In severe hemophilia, prophylactic
replacement therapy is given on
regular basis to prevent chronic
arthropathy.
Inhibitors are IgG against infused factors ( 8>9). It occurs in up to 15% of patients with severe factor 8 deficiency. العلاج / frequent steroid Desmopressin (DDAVP) can be used instead of replacement therapy in mild to moderate hemophilia A (Not Hemophilia B). • It is given by infusion and stimulates endogenous release of FVIII:C and vWF.
von Willebrand Disease
von Willebrand Disease
• Usually Autosomal Dominant. • 1% of population • Caused by deficiency in von Willebrand
factor (vWF). • vWF function: ü Facilitates platelet adhesion. üActs as Factor 8 carrier protein. • vWF deficiency can be quantitative or
qualitative.
Ma nagement
• DDAVP Causes secretion of factor 8 and vWF into plasma. • Plasma-Derived Factor 8 in severe
cases. • Avoidance of anticoagulants, NSAIDs
and IM injections.
Hemorrhagic disease of the newborn
Due to vitamin K deficiency which is
essential for coagulation factors
production. • Risk is increased in breast fed infants. • 1-8 weeks of age. • Bleeding can vary between mild to
severe. • All newborns should receive Vitamin K
at birth.
Immune Throbocytopenic Purpura (ITP)
Immu ne Throbocytopenic Purpura (ITP)
• The most common cause of
thrombocytopenia in children. • It is an autoimmune disease caused by
autoantibodies against platelets.
• 2-10 years of age. • The bleeding is usually preceded by a
viral infection. • Petechiae, purpura, or superficial
bruising. • Can cause mucus membrane bleeding
such as epistaxis. • Intracranial hemorrhage is uncommon
despite the lower platelets.
ITP is a diagnosis of exclusion. • Any atypical features such as anemia,
leukocytopenia, hepatosplenomegaly
or marked lymphadenopathy should
raise the suspicion for possible
malignant process.
• CBC: Low platelets, normal RBC and
WBC counts. • Bone morrow examination is not
necessary unless there is atypical
history, physical examination or initial
workup. • BM (if done) reveals increased
megakaryocytes and normal erythroid
and myeloid elements.
For children with no active bleeding: - Observation (First line treatment) - Oral steroid - Intravenous Immunoglobulin (IVIG) • For children with moderate bleeding: - IVIG or short course of corticosteroid • For children with life-threatening
bleeding: - Intravenous steroids and IVIG, may consider platelet transfusion
Chronic ITP ITP that persists for 12 months is classified as chronic ITP. • Secondary causes of chronic ITP, especially SLE and HIV infection, should be ruled out.
Wiskott-Aldrich syndrome
Wiskott-Aldrich syndrome
• X-linked recessive. • Triad of immune deficency, eczema,
and thrombocytopenia. • Hematopoietic stem cell
transplantation cures the
immunodeficiency and
thrombocytopenia.
