• Hypochromic + microcytic anememia (might be normocytic initially)
• low serum iron
• low ferritin
• incr. TIBC + Transferrin
• Normal/Low Reticulocytes
• Thrombocytosis
• high RDW
• 🦴-marrow → Absent iron reserves (Perl's stain)

• Has to present decreased MCV and MCH Values in the complete blood count
• Can be a Thalassemia, Anemia of a chronic Disease, Iron deficiency anemia or sideroblastic anemia

• Low serum iron
• Low TIBC
• Normal/high ferritin (elev. due to inflammation) -associated with elevation in acute phase reactants (ESR, CRP, fibrinogen, platelets)

Heterozygotes (minor beta talassemia)

• normal clinical status
• mild pallor
• hepatosplenomegaly (HSM)

Homozygotes (major beta talassemia)

• growth retardation
• bone deformeties - short limbs, mongoloid facies
• intense pallor, jaundice + melanodermia (pseudo-tanned)
• HSM
• Iron overload! → organe dmg (HF, LF, hypogondism, DM,RF)

• severe microcytic anemia
• target cells, basophilic inclusion
• teardrop cells
• Erythroblasts in periph blood
• indirect hyperbili
• normal-high iron, ferritin, transferrin
• Perls stain → incr. hemosiderin in target organs
• low RDW
• Electrophoresis! → low HbA1, incr. HbA2 + HbF

Hereditary: Bernard-Soulier (Gp1b), Glanzmanns (G2b-3a), storage pool disease, (vWD if associated with FVIII); or vWD Type 2

Acquired: drugs (NSAIDS, ASS), Uremia, myeloproliferative disorders

• Splenomegaly due to:
• TTP, Hemolytic Uremic Snd, Heparin induced Thrombocytopenia, DIC
• ITP → Idiopathic + secondary

→idiopathic children; second. adults

• idiopathic: auto-AB platelet destruction
• secondary→Antibiotics, Lupus, Aids, Hep C
• classic clinical picture:

MUCO-CUTANEOUS BLEEDING

1. petechia, echymosis, purpura
2. epistaxis
3. gum bleeding
4. no splenomegaly

• Steroids (1-2mg/kg)
• Splenectomy (if no response to steroids)
• IVIG
• Thrombopoietin receptor agonist (Eltrombopag + Romiplostim)

• abrupt onset
• mucocutaneous bleeding
• pallor
• jaundice
• thrombotic events (cerebral, cardiac)
• neuro-psychiatric symptoms
• renal failure

LAB

• thrombocytopenia
• hemolytic anemia
• reticolocytosis
• Schisocytes (red helmet cells)
• BUN+Crea elev.
• Low ADAMTS13 + AB

• muco-cutaneoal bleeding (esp. Ear-nose-throat)
• prologend BT
• normal PT + APTT (→ APTT high if low FVIII)
• low vWF
• Ristocetin cofactor activity → neg. in vWD

Hemophilia A → FVIII

Hemophilia B → F IX

deep bleeding:

• hemarthrosis (knee, shoulder, elbow, hip)
• hematomas
• hematuria

severe →spontaneous hemorrh (<1% residual factor)

medium → after minor trauma (1-5%)

mild → after trauma or surgery (5-25%)

⇒ DG

• incr. APTT
• normal PT, ThrombinT, BT

Causes:

"STOP Making Trouble"

• infections (sepsis)
• trauma
• Obstetric complications
• Pancreatitis
• malignancies (acute leukemia)
• Acute hemolytic transfusion reaction

Lab:

• increased aPTT, PT + bleeding time
• high D-DImers,
• low Fibrinogen
• Thrombocytopenia
• Schistocytes