'Myelokathexis': Neutropenia With Narrow Hyperplasia
'Myelokathexis': Neutropenia With Narrow Hyperplasia
O'Regan, Sean;Newman, Arthur J.;Graham, Richard C.
1977-06-01 00:00:00
Abstract • A 10-year-old girl was first seen at age 5 years with pneumonia and neutropenia. Since then, she has remained leukopenic, although manifesting a leukocytosis only when she has pulmonary infection. A rapid fall in her peripheral WBC count occurs with initiation of antibiotic therapy. Despite her neutropenia, marked myeloid hyperplasia is evident on marrow smear examination; many cells being hypersegmented with fine intralobular bridging with chromatin strands and cytoplasmic vacuolation. The peripheral WBC response to epinephrine administration did not indicate a shift from the circulating to marginal neutrophil pool. Results from a Rebuck skin window test suggested poor neutrophil tissue migration. A defect in granulocyte release from the patient's marrow may explain these bizarre hematologic findings. (Am J Dis Child 131:655-658, 1977) References 1. Baehner RL: Disorders of leukocytes leading to recurrent infection . Pediatr Clin North Am 19:935-956, 1972. 2. Krill CE, Smith HD, Mauer AM: Chronic idiopathic granulocytopenia . N Engl J Med 270:973-979, 1964.Crossref 3. Zuelzer WW: "Myelokathexis": A new form of chronic granulocytopenia . N Engl J Med 270:699-704, 1964.Crossref 4. Polmar SM, Chase PA: Quantitation of the biosynthesis of immunoglobulin in peripheral blood lymphocytes of normal and immunodeficient patients . J Pediatr 87:545-549, 1975.Crossref 5. Page AR, Good RA: Studies on cyclic neutropenia: A clinical and experimental investigation . Am J Dis Child 94:623-661, 1957. 6. Schwachman M, Diamond LK, Oski FA, et al: The syndrome of pancreatic insufficiency and bone marrow dysfunction . J Pediatr 65:645-663, 1964.Crossref 7. Zuelzer WW, Bajoghl M: Chronic granulocytopenia in childhood . Blood 23:359-374, 1964. 8. Rothstein G, Hugh E, Bishop C, et al: Stimulation of granulocytopoiesis by a diffusible factor in vivo . J Clin Invest 50:2004-2007, 1971.Crossref 9. Boggs DR, Marsh JC, Chervenick PA, et al: Neutrophil releasing activity in plasma of normal human subjects injected with endotoxin . Proc Soc Exp Biol Med 127:689-693, 1968. 10. Greenberg PL, Schrier SL: Clinical utility of in vitro evaluation of granulopoiesis . Ann Rev Med 25:269-278, 1974.Crossref 11. Boggs DR, Cartwright GE, Wintrobe MM: Neutrophilia inducing activity in plasma of dogs recovering from drug induced myelotoxicity . Am J Physiol 211:51-60, 1968. 12. Marsh JC, Levitt M: Neutrophilia inducing activity in plasma of neutropenic human beings . Blook 37:647-656, 1971.
http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.pngAmerican Journal of Diseases of ChildrenAmerican Medical Associationhttp://www.deepdyve.com/lp/american-medical-association/myelokathexis-neutropenia-with-narrow-hyperplasia-dWEwJRu0DQ
'Myelokathexis': Neutropenia With Narrow Hyperplasia
Abstract • A 10-year-old girl was first seen at age 5 years with pneumonia and neutropenia. Since then, she has remained leukopenic, although manifesting a leukocytosis only when she has pulmonary infection. A rapid fall in her peripheral WBC count occurs with initiation of antibiotic therapy. Despite her neutropenia, marked myeloid hyperplasia is evident on marrow smear examination; many cells being hypersegmented with fine intralobular bridging with chromatin strands and cytoplasmic vacuolation. The peripheral WBC response to epinephrine administration did not indicate a shift from the circulating to marginal neutrophil pool. Results from a Rebuck skin window test suggested poor neutrophil tissue migration. A defect in granulocyte release from the patient's marrow may explain these bizarre hematologic findings. (Am J Dis Child 131:655-658, 1977) References 1. Baehner RL: Disorders of leukocytes leading to recurrent infection . Pediatr Clin North Am 19:935-956, 1972. 2. Krill CE, Smith HD, Mauer AM: Chronic idiopathic granulocytopenia . N Engl J Med 270:973-979, 1964.Crossref 3. Zuelzer WW: "Myelokathexis": A new form of chronic granulocytopenia . N Engl J Med 270:699-704, 1964.Crossref 4. Polmar SM, Chase PA: Quantitation of the biosynthesis of immunoglobulin in peripheral blood lymphocytes of normal and immunodeficient patients . J Pediatr 87:545-549, 1975.Crossref 5. Page AR, Good RA: Studies on cyclic neutropenia: A clinical and experimental investigation . Am J Dis Child 94:623-661, 1957. 6. Schwachman M, Diamond LK, Oski FA, et al: The syndrome of pancreatic insufficiency and bone marrow dysfunction . J Pediatr 65:645-663, 1964.Crossref 7. Zuelzer WW, Bajoghl M: Chronic granulocytopenia in childhood . Blood 23:359-374, 1964. 8. Rothstein G, Hugh E, Bishop C, et al: Stimulation of granulocytopoiesis by a diffusible factor in vivo . J Clin Invest 50:2004-2007, 1971.Crossref 9. Boggs DR, Marsh JC, Chervenick PA, et al: Neutrophil releasing activity in plasma of normal human subjects injected with endotoxin . Proc Soc Exp Biol Med 127:689-693, 1968. 10. Greenberg PL, Schrier SL: Clinical utility of in vitro evaluation of granulopoiesis . Ann Rev Med 25:269-278, 1974.Crossref 11. Boggs DR, Cartwright GE, Wintrobe MM: Neutrophilia inducing activity in plasma of dogs recovering from drug induced myelotoxicity . Am J Physiol 211:51-60, 1968. 12. Marsh JC, Levitt M: Neutrophilia inducing activity in plasma of neutropenic human beings . Blook 37:647-656, 1971.
Journal
American Journal of Diseases of Children
– American Medical Association
Published: Jun 1, 1977
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References
Disorders of leukocytes leading to recurrent infection
Baehner RL
Chronic idiopathic granulocytopenia
Krill CE, Smith HD, Mauer AM
A new form of chronic granulocytopenia
Zuelzer WW: "Myelokathexis"
Quantitation of the biosynthesis of immunoglobulin in peripheral blood lymphocytes of normal and immunodeficient patients
Polmar SM, Chase PA
Studies on cyclic neutropenia: A clinical and experimental investigation
Page AR, Good RA
The syndrome of pancreatic insufficiency and bone marrow dysfunction
Schwachman M, Diamond LK, Oski FA, et al
Chronic granulocytopenia in childhood
Zuelzer WW, Bajoghl M
Stimulation of granulocytopoiesis by a diffusible factor in vivo
Rothstein G, Hugh E, Bishop C, et al
Neutrophil releasing activity in plasma of normal human subjects injected with endotoxin
Boggs DR, Marsh JC, Chervenick PA, et al
Clinical utility of in vitro evaluation of granulopoiesis
Greenberg PL, Schrier SL
Neutrophilia inducing activity in plasma of dogs recovering from drug induced myelotoxicity
Boggs DR, Cartwright GE, Wintrobe MM
Neutrophilia inducing activity in plasma of neutropenic human beings
Marsh JC, Levitt M
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