By Eric D. Hsi
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In general, serum ferritin level less than 100 mg/L (in nondialyzed patients) or transferrin saturation less than 20%, or both, is considered compatible with iron deficiency in patients with chronic kidney disease. Even more challenging is the detection of the functional iron deficiency that may compromise the effectiveness of ESA therapy. The small numbers of hypochromic RBCs produced in this circumstance will not be detected reliably by the MCV or MCHC. Parameters to detect functional iron deficiency include percentage of hypochromic 17 CHAPTER 1â•… Red Blood Cell/Hemoglobin Disorders FIGURE 1-12â•… Renal osteodystrophy, bone marrow biopsy findings.
Differential Diagnosis The differential diagnosis of SCD includes other forms of chronic hemolytic anemia. However, only SCD is associated with sickled RBCs and vasoocclusive phenomena. Hb SC disease can be confused with Hb CC disease because of the presence of Hb C crystals. However, the crystals in CC disease are straight, in contrast to the irregular, bent, or malformed crystals of SC disease. Rare cases of SS disease will have few sickled cells but frequent blister cells. Such cases may be confused with oxidant hemolysis.
Splenectomized patients are at risk of postsplenectomy sepsis, which can be fatal. Hereditary Elliptocytosis and Hereditary Pyropoikilocytosis Hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis (HPP) are related disorders caused by defects in the horizontal interactions between membrane cytoskeletal proteins, specifically the association of spectrin heterodimers into heterotetramers. Like HS, these are genetically and clinically heterogeneous. HE results from a heterozygous gene defect, whereas HPP results from homozygosity for a particular HE mutation, double heterozygosity for two different HE mutations, or double heterozygosity for an HE mutation and a lowexpression α-spectrin allele.
Hematopathology by Eric D. Hsi