Internal Medicine

0521779407-13 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:15

Leukocytosis: Neutrophil Leukopenia 901

➣Low platelets, hematocrit suggestive of primary marrow failure

syndrome or hematopoietic malignancy

■In patient with fever and ANC<500, evaluation should include cul-

tures of blood and urine, chest xray.

Bone Marrow Examination

■Most patients require bone marrow examination

■With accompanying anemia, thrombocytopenia: R/O apla-

sia, leukemia, myelodysplasia, other primary marrow malig-

nancy

■Hyperplastic myeloid precursors and a “maturation arrest”: suggests

peripheral neutrophil destruction, as seen in collagen vascular dis-

ease or drug-induced neutropenia

differential diagnosis

Decreased Production of Neutrophils

■Congenital syndromes

➣Congenital agranulocytosis (Kostmann’s syndrome)

Usually autosomal recessive, but also autosomal dominant

and sporadic cases

Early onset, frequent, life-threatening infections with severe

neutropenia

Bone marrow aspirate: maturation arrest at the promyelocyte

stage.

Responds to G-CSF

Responds to hematopoietic cell transplantation

Increased incidence of AML and MDS

Linked to mutations in neutrophil elastase, a primary granule

protein

➣Benign cyclic neutropenia (cyclic hematopoiesis)

Rare dominantly inherited marrow disorder

Characterized by cyclic fluctuations in neutrophil counts in

21 day cycle

Episodes of neutropenia severe, ANC <200× 106 /microliter;

may be accompanied by fevers, pharyngitis, stomatitis, and

other bacterial infections.

Also linked to the neutrophil elastase gene

Unlike Kostmann’s syndrome, no increased incidence of AML

and MDS.