Sideroblastic anemia. Presence of anemia + ringed sideroblasts. Hypochromic, BIMODAL RBC population. +/- Pappenheimer bodies (iron). Increased iron stores in BM.  All iron studies are elevated (but distinguish from hemochromocytosis because SA has low hgb and low MCV). CAUSES: ACQUIRED = RARS, meds, alcohol, Pearson syndrome and low copper. VAST MAJORITY HAVE CLONAL DEFECT. MACROCYTIC. >15% RS. INHERITED = rare, X-linked. ALAS2 gene. MICROCYTIC. Can try giving a ton of B6.

Sideroblastic anemia. Presence of anemia + ringed sideroblasts. Hypochromic, BIMODAL RBC population. +/- Pappenheimer bodies (iron). Increased iron stores in BM. All iron studies are elevated (but distinguish from hemochromocytosis because SA has low hgb and low MCV). CAUSES: ACQUIRED = RARS, meds, alcohol, Pearson syndrome and low copper. VAST MAJORITY HAVE CLONAL DEFECT. MACROCYTIC. >15% RS. INHERITED = rare, X-linked. ALAS2 gene. MICROCYTIC. Can try giving a ton of B6.

Pappenheimer (e.g. in sideroblastic anemia) vs. Howell-jolly body (e.g. in sickle cell)

Pappenheimer (e.g. in sideroblastic anemia) vs. Howell-jolly body (e.g. in sickle cell)

Special Types Of Anemia: Sideroblastic Anemias And Paroxysmal Nocturnal Hemoglobinuria (PNH)

Special Types Of Anemia: Sideroblastic Anemias And Paroxysmal Nocturnal Hemoglobinuria (PNH)

Sideroblastic anemia - Causes, Diagnosis, Treatment, Ongoing care and Clinical pearls

Pappenheimer Bodies = iron granules (sideroblastic anemia)

Pappenheimer Bodies = iron granules (sideroblastic anemia)

Pappenheimer bodies = IRON granules in mitochondria = sideroblastic anemia and asplenism.

Pappenheimer bodies = IRON granules in mitochondria = sideroblastic anemia and asplenism.

A ringed sideroblast must have 10 siderosomes surroundng at least one-third the nucleus.  Can be seen in ETOH, pyrazinamide, lead and benzene.

A ringed sideroblast must have 10 siderosomes surroundng at least one-third the nucleus. Can be seen in ETOH, pyrazinamide, lead and benzene.

Ringed sideroblast sideroblastic anemia

Ringed sideroblast sideroblastic anemia

sideroblastic anemia = ringed sideroblasts in BM; low MCHC, bimodal RDW; iron indices all high (serum iron, transferrin, ferritin)

sideroblastic anemia = ringed sideroblasts in BM; low MCHC, bimodal RDW; iron indices all high (serum iron, transferrin, ferritin)

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