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1090 Part VIII: Monocytes and Macrophages Chapter 69: Disorders of Monocytes 1091

TABLE 69–1. Disorders of Monocytes and Macrophages
I. Monocytopenia VI. Clonal (Neoplastic) Histiocytosis (Chap. 71)
A. Aplastic anemia 1 A. Langerhans cell histiocytosis 41,42
B. Hairy cell leukemia 2 (1) Localized
C. MonoMAC syndrome 3–7 (2) Systemic
D. Glucocorticoid therapy 8,9 B. Tumors or sarcomas of histiocytes and dendritic cells 43
II. Monocytosis (1) Histiocytic sarcoma
A. Benign (2) Langerhans cell sarcoma
(1) Reactive monocytosis 10 (3) Interdigitating dendritic cell sarcoma
(2) Exercise-induced 11 (4) Follicular dendritic cell sarcoma
B. Clonal monocytosis VII. Monocyte and Macrophage Dysfunction 44–46
Indolent A. α -Proteinase inhibitor deficiency 47,48
1
(1) Chronic idiopathic monocytosis 12 B. Chédiak-Higashi syndrome 49
(2) Oligoblastic myelogenous leukemia (myelodysplasia) 13 C. Chronic granulomatous disease 50,51
Progressive D. Chronic lymphocytic leukemia 52,53
(1) Acute monocytic leukemia 14–16 E. Disseminated mucocutaneous candidiasis 54,55
(2) Dendritic cell leukemia 17–19 F. Glucocorticoid therapy 56,57
(3) Progenitor cell monocytic leukemia 20 G. Kawasaki disease 58,59
(4) Chronic myelomonocytic leukemia 21,22 H. Malakoplakia 60
(5) Juvenile myelomonocytic leukemia 23 I. Mycobacteriosis syndrome 61–63
III. Macrophage Deficiency J. Leprosy 64
A. Osteopetrosis (isolated osteoclast deficiency) 24,25 K. Posttraumatic 65,66
IV. Inflammatory Histiocytosis (Chap. 71) L. Septic shock-induced 67–69
A. Primary hemophagocytic lymphohistiocytosis 26–28 M. Critically ill subjects 70
(1) Familial N. Solid tumors 71,72
(2) Sporadic O. Tobacco smoking 73,74
B. Other inherited syndromes with hemophagocytosis lym- P. Marijuana smoking or cocaine inhalation 75,76
phohistiocytosis: Chédiak-Higashi, X-linked lymphoprolifer- Q. Whipple disease 77,78
ative, Gracelli 29 R. Human interleukin (IL)-10 effects; Epstein-Barr virus
C. Infectious hemophagocytic histiocytosis 30,31 IL-10–like gene product (vIL-10) 79,80
D. Tumor-associated hemophagocytic histiocytosis 31,32 VIII. Atherogenesis 81–85
E. Drug-associated hemophagocytic histiocytosis 33 IX. Thrombogenesis 85–88
F. Disease-associated hemophagocytic histiocytosis 29–32 X. Obesity 89
G. Juvenile rheumatoid arthritis (macrophage activation XI. Aging 90–92
syndrome) 33,34
H. Sinus histiocytosis with massive lymphadenopathy 35,36
V. Storage Histiocytosis (Chap. 72)
A. Gaucher disease 37
B. Niemann-Pick disease 38
C. Gangliosidosis 39
D. Sea-blue histiocytosis syndrome 40

cells, committed to the monocyte-macrophage lineage, can undergo hybrid disease because of the association with an underlying mono-
malignant transformation (Chaps. 83 and 88). clonal or oligoclonal proliferation of virus-infected lymphocytes. The
Several uncommon types of histiocytosis are serious systemic striking activation of macrophages and the resulting cytokine elabora-
diseases that may masquerade as malignant disease. However, in such tion and organ pathology seen in some patients with juvenile rheuma-
cases the cytopathologic changes in monocytes or macrophages do not toid arthritis, referred to as the “macrophage-activation syndrome,” is
constitute a malignant transformation and are not monoclonal. Familial closely related to other types of hemophagocytic syndromes (Chap. 71).
and sporadic hemophagocytic lymphohistiocytosis, infection-induced Pediatric rheumatologists refer to the hemophagocytic syndrome in
hemophagocytic syndromes, and sinus histiocytosis with massive patients with juvenile rheumatoid arthritis as the “macrophage acti-
lymphadenopathy are among such disorders (Chap. 71). Infectious vation syndrome,” but the clinical expression is closely analogous to
hemophagocytic histiocytosis caused by Epstein-Barr virus may be a other acquired hemophagocytic lymphohistiocytic syndromes. In these

Kaushansky_chapter 69_p1089-1094.indd 1090 9/18/15 9:47 AM