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1098 Part VIII: Monocytes and Macrophages Chapter 70: Monocytosis and Monocytopenia 1099

BLOOD MONOCYTE SUBSET COUNTS or with acute myelogenous leukemia. Because of the GATA2 gene
123
product’s role in the development of the vascular and lymphatic sys-
IN DISEASE tems, some cases may have the triad of lymphedema, monosomy 7,
and myelodysplasia or acute myelogenous leukemia, designated the
Differential monocyte subset responses (CD14 CD16 − vs. Emberger syndrome. 124–127 Hematopoietic stem cell transplantation has
++
CD14 CD16 ) without deviation of total monocyte counts outside been successful in restoring normal immunohematopoiesis in some of
+
+
the normal range have been observed in older subjects and those with the patients so treated. 128
sepsis, AIDS, allergic disorders, dermatitides, hemodialysis, and ath- Glucocorticoid hormones produce a monocytopenia, transiently,
erosclerosis. 4,10,91,109 These monocytic subset variations usually are not approximately 6 hours after administration to human volunteers 129,130
measured in clinical laboratories and, as yet, have little diagnostic or or to patients. Administration of interferon-α and tumor necrosis
95
prognostic importance.
factor-α may also cause monocytopenia. Monocytopenia may follow
131
radiotherapy. 132
DISORDERS ASSOCIATED WITH
MONOCYTOPENIA BLOOD DENDRITIC CELL COUNTS
Table 70–2 lists the disorders associated with monocytopenia. Blood dendritic cells are composed of two phenotypic subtypes: mye-
+
+
+
Although monocytopenia may occur in any hematopoietic multipoten- loid-derived (HLA-DR CD11c CD123 ) and lymphoid-plasmacytoid-
+
+
−
tial cell disease associated with pancytopenia (e.g., acute myelogenous derived (HLA-DR CD11c CD123 ). The total blood dendritic cell
leukemia), a decrease in monocytes is notable and constant in aplas- count can be measured by flow cytometry. 133–135 Dendritic cells make
tic anemia and hairy cell leukemia, in which monocytopenia can up approximately 0.6 percent of blood cells (range: 0.15 to 1.30 percent)
110
111
6
6
be a helpful diagnostic clue and also a contributor to the predisposi- and represent 14 × 10 cells/L (range: 3 to 30 × 10 cells/L). Approx-
tion to infection, which is an important, morbid feature of the disease. imately one-third of these cells are a lymphoid-plasmacytoid–derived
Monocytopenia occurs in a small proportion of patients with chronic type and two-thirds are a myeloid-derived type. 135–137 Fluctuations in
lymphocytic leukemia and these patients may have a higher frequency blood dendritic cells are often independent of changes in total blood
138
of infections, especially by viruses. Severe thermal injuries also can monocyte count. Blood dendritic cell counts decrease with aging and
112
137
result in monocytopenia. Cyclic neutropenia is also notable for inter- increase with surgical stress (and presumably other stressful reac-
113
mittent periods of monocytopenia. Rare cases of conjoint severe neu- tions) in relation to plasma cortisol levels.
114
tropenia and monocytopenia occur. Transient monocytopenia is a
115
feature of hemodialysis, but monocyte counts return to normal within REFERENCES
hours after the procedure ends. 109
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thematosus, and in those with human immunodeficiency virus infec- subpopulation. Immunol Today 17:424, 1996.
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inflammatory monocyte as biomarker for inflammatory diseases. Biomark Res 2:1, 2014.
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the GATA2 gene that decrease transcription of the gene message. It 10. Sadeghi HM, Schnelle JF, Thoma JK, et al: Phenotypic and functional characteristics of
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may present as an atypical type of MDS with a hypocellular marrow, but circulating monocytes of elderly persons. Exp Gerontol 34:959, 1999.
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TABLE 70–2. Disorders Associated with Monocytopenia 13. Cunningham I, MacCallum SJ, Nicholls MD, et al: The myelodysplastic syndromes:
An analysis of prognostic factors in 226 cases from a single institution. Br J Haematol
I. Any cause of severe leukopenia 90:602, 1995.
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Cancer Treat Res 108:81, 2001.
B. Hairy cell leukemia 111 15. Jaworkowsky LI, Solovey DY, Rhausova LY, Udris OY: Monocytosis as a sign of subse-
C. Other myeloid or lymphoid malignancies resulting in sup- quent leukemia in patients with cytopenias (preleukemia). Folia Haematol Int Mag Klin
pression of monocytopoiesis Morphol Blutforsch 110:395, 1983.
II. MonoMAC syndrome 120–123 and Emberger syndrome 124,125 16. Ruggiero G, Sica M, Luciano L, et al: A case of myelodysplastic syndrome associated
with CD14(+)CD56(+) monocytosis, expansion of NK lymphocytes and defect of
(GATA2 gene mutations) HLA-E expression. Leuk Res 33:181, 2009.
III. Miscellaneous conditions (see section “Disorders Associated 17. Boiocchi L, Espinal-Witter R, Geyer JT, et al: Development of monocytosis in patients
with primary myelofibrosis indicates an accelerated phase of the disease. Mod Pathol
with Monocytopenia”) 26:204, 2013.
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