CD110

Aka thrombopoietin receptor (TPO-R), myeloproliferative leukemia virus oncogene (MPL)
Binding to thrombopoietin induces megakaryocyte proliferation and differentiation and prevents apoptosis
Mutations found in some patients with congenital amegakaryocytic thrombocyenia, a disease characterized by severe congenital thrombocyenia with variable bleeding tendency and absence of megakaryocytes in the bone marrow
Positive staining (normal): hemopoietic stem and progenitor cells, megakaryocytes and progenitors, platelets
 

CD111

Aka poliovirus receptor related 1 (PRR1), nectin1
Widely expressed adhesion molecule that is a component of the adherens junction; receptor for herpes simplex viruses 1 and 2
Positive staining (normal): cells from myeloid, monocyte, megakaryocytes and erythroid lineage, epithelial cells, neurons, endothelium
 

CD112

Aka poliovirus receptor related 2 (PRR2)
Adhesion molecule that is a component of the adherens junction; receptor for herpes simplex virus
Positive staining (normal): cells from myeloid, monocyte, megakaryocytes lineage, epithelial cells, neurons, endothelium
 

CD113

Reserved for future use
 

CD114

Aka granulocyte colony-stimulating factor receptor, G-CSFR
Specific regulator of myeloid proliferation and differentiation
Mutations present in some patients with severe congenital neutropenia
Positive staining (normal): granulocytes (all), monocytes, platelets, endothelium, placenta, trophoblastic cells
Positive staining (tumors): cultured tumor cells
Negative staining: eosinophils, lymphocytes, erythrocytes
 

CD115

Aka c-fms, receptor for macrophage colony stimulating factor, CSF-1R
Localized on cylasmic membrane; mediates biologic activity of CSF-1
v-fms is a viral oncogene present in the feline McDonough sarcoma virus (has several substitute mutations)
Related to platelet derived growth factor receptor, c-kit, vascular endothelial growth factor receptor, fibroblast growth factor receptor, high affinity nerve growth factor (TrkA)
Deficiency causes reduced osteoclasts and macrophages, abnormal bone remodeling and osteopetrosis, abnormal breast development and decreased fertility
Positive staining (normal): macrophages and precursors, osteoclasts, placental trophoblast, breast tissue, microglia, neurons, astrocytes
Positive staining (tumors): 10% AML, some endometrial, ovarian and breast cancers, vascular smooth muscle cells in atheromas, choriocarcinoma cells
 

CD116

GM-CSF receptor alpha chain
Primary binding subunit of GM-CSF receptor
Positive staining (normal): monocytes, neutrophils, eosinophils, fibroblasts, endothelial cells, dendritic cells
 

CD117

Summary: proto-oncogene activated in GIST tumors
Aka c-kit, stem cell factor receptor
Gene at 4q11-21
Receptor for kit protein, a 145 kD tyrosine kinase growth factor receptor protein important for development and survival of mast cells, hemopoietic stem cells, melanocytes, germ cells, interstitial cells of Cajal
Has activating or gain of function mutations in most GIST tumors, often at exon 11, less often at exons 9 and 13, Hum Path 2002;33:484
Molecular images (Hum Path subscribers): image1
Kit signaling pathways (Hum Path subscribers): chart1
Tyrosine kinase activity of c-kit in GIST and bcl-abl overexpression in CML are inhibited by imatinib mesylate (Gleevec, STI571), a tyrosine kinase inhibitor used to treat these diseases, Hum Path 2002;33:466
Uses: confirming diagnosis of GIST (but see other CD117+ tumors below), possibly confirm chronic intestinal pseudo-obstruction, AJSP 2003;27:228
Interpretation: should be strong and diffuse cylasmic staining, like the positive control
Positive staining (normal): interstitial cells of Cajal, hemopoietic progenitor cells, melanocytes, embryonic/fetal brain, endothelium, gonads, mast cells, breast epithelium, germ cells
Positive staining (tumors): AML, angiomyolipoma (AJSP 2002;26:493), angiosarcomas (50%), clear cell sarcoma, CML, epithelioid sarcoma, Ewing sarcoma, gastrointestinal autonomic tumors, gastrointestinal stromal tumors (GIST), granulocytic sarcoma, Hodgkin’s lymphoma (some Reed-Sternberg cells), intraabdominal fibromatosis (depends on antibody used, AJSP 2001;25:549), mast cell disease (also positive for tryptase, CD43, CD68); melanoma, mesenteric fibromatosis (variable, AJSP 2002;26:1296), metanephric adenosarcoma (AJSP 2001;25:1451), omental mesenchymal tumor, osteosarcoma, rhabdomyosarcoma, sclerosing mesenteritis (variable), seminomas /dysgerminomas, small cell lung cancer (Hum Path 2002;33:1182), synovial sarcoma (~10%, usually cylasmic staining), adenoid cystic carcinoma of salivary gland (strong staining)
Negative staining: alveolar soft part sarcomas, desmoplastic small round cell tumors, glomus tumors, leiomyomas (retroperitoneal, colorectal), leiomyosarcomas, myxomas (cardiac), schwannomas  (colorectal), smooth muscle tumors of uncertain malignant potential, solitary fibrous tumors
Micro images: rhabdomyosarcoma of pulmonary trunk, GIST vs. solitary fibrous tumor, malignant stromal tumor of gallbladder (figure 4),
Micro images (AJSP subscribers): GIST (site unspecified), rectal GIST (figure A), metanephric adenosarcoma of kidney (stroma only), normal small bowel (figure 1A), normal colon (figure 2A), chronic intestinal pseudo-obstruction (figure 3A), intraabdominal fibromatosis (variable results with different antibodies)- #1, #2, pediatric synovial sarcomas, pediatric osteosarcomas, pediatric Ewing’s/PNET
Micro images (Hum Path subscribers): GIST #1, #2, #3, #4 (figure A), small cell lung cancer of lung (A: H&E, B: control-GIST, C: 2+ staining, D: 1+ staining)
Micro images (Mod Path subscribers): GIST (figures G, H), interstitial cells of Cajal in patient with multiple GIST tumors
References: Archives 2002;126:1189, AJSP 2002;26:486
 

CD118

Reserved for interferon alpha / gamma receptor
 

CDw119

Interferon gamma receptor
Positive staining (normal): macrophages, B cells
 

CD120a

Aka TNFR1, receptor for tumor necrosis factor alpha and beta, type I
Receptor binding to ligand causes apoptosis
 

CD120b

Aka receptor for tumor necrosis factor (TNF) alpha and beta, type II
 

CD121a

Aka Interleukin 1 receptor (IL-1R) type I, IL1-alpha
Interleukin-1, an inflammatory mediator, consists of 2 separate but related proteins, IL1-alpha and IL1-beta
Positive staining (normal): T cells, thymocytes, fibroblasts, endothelial cells
 

CDw121b

Aka Interleukin 1 receptor (IL-1 R) type II, IL1-beta
May inhibit IL1 activity by acting as a decoy target for IL1
Positive staining (normal): B cells, macrophages
 

CD122

Aka Interleukin 2 receptor beta chain (IL-2R beta)
Critical component of IL-2 and IL-15-mediated signaling
Positive staining (normal): NK cells, B cells, T cells, monocytes
 

CD123

Aka Interleukin 3 receptor alpha subunit
Associates with the GM-CSF receptor (GMR beta)
Positive staining (normal): plasmacytoid monocytes (bright with flow cytometry, AJSP 2002;26:852)
 

CD124

Aka Interleukin 4 receptor
Receptor subunit for Interleukin-4 and 13
Positive staining (normal): mature B cells, T cells; hemopoietic precursors; fibroblasts, endothelial cells
 

CDw125

Aka Interleukin 5 receptor alpha subunit
Therapeutic target of eosinophilic inflammation involved in bronchial asthma
Positive staining (normal): eosinophils, activated B cells, basophils
 

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