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2.
Dermatol Online J. 2017 Aug 15;23(8). pii: 13030/qt6zx243zm.

Telangiectasia macularis eruptiva perstans: an old terminology, still frequently used.

Author information

1
John P. and Kathrine G. McGovern Medical School at the University of Texas Health Science Center, Houston, Texas Department of Dermatology, The University of Texas, MD Anderson Cancer Center, Houston, Texas. Kelly.M.Wilmas@uth.tmc.edu.

Abstract

The term telangiectasia macularis eruptiva perstans (TMEP) was originally used to describe a rare form of cutaneous mastocytosis (CM) that was limited to the skin with lesions consisting of irregular, telangiectatic macules ranging in color from red to brown. Recent guidelines, however, recommended that the sole presence of telangiectasias should not form the basis of a distinct variant of CM. We conducted a review of the literature from 1930 to 2017 and found 76 cases that were reported as TMEP. Owing to a general misconception about diagnosis of CM and SM, there is a need for further discussion and awareness of the newly proposed World Health Organization (WHO) guidelines.

PMID:
29469737
[Indexed for MEDLINE]
3.
Diagn Pathol. 2018 Feb 20;13(1):14. doi: 10.1186/s13000-018-0691-2.

De novo mast cell leukemia without CD25 expression and KIT mutations: a rare case report in a 13-year-old child.

Author information

1
Department of Pathology, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China.
2
Department of Pathology, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China. 06510@pkufh.cn.

Abstract

BACKGROUND:

Mast cell leukemia (MCL) is a very rare form of systemic mastocytosis (SM) and accounts for less than 0.5% of all mastocytosis. The diagnosis of MCL requires the presence of SM criteria, accompanied by leukemic infiltrating of atypical mast cells (MCs) in bone marrow (BM), peripheral blood as well as extracutaneous organs. MCL is a fatal disease that almost always behaves aggressively, and the median survival time is only about six months. Herein, we present a rare case of de novo MCL without CD25 expression and KIT mutations.

CASE PRESENTATION:

A previously healthy 13-year-old boy was referred to our hospital due to incidental discovery of an enlarged right tonsil. Diffuse infiltration of medium-sized hematopoietic blasts was found in his right tonsil, BM and multiple lymph nodes. The neoplastic cell population was subsequently revealed to exhibit differentiation towards the mast cell lineage by expressing CD117 and tryptase, but the cell population lacked expression of CD25/CD2 and the activating mutation of the KIT gene. An abnormal karyotype was identified, but no leukemia-associated fusion genes were found. Involvement of peripheral blood, bone and lung was subsequently demonstrated. The most important differential diagnosis included tryptase-positive (T+) acute myeloid leukemia, myelomastocytic leukemia and basophilic leukemia. The morphological characteristics and infiltrating patterns of the abnormal MCs supported the final diagnosis of MCL. Although intensive chemotherapy and allogeneic stem cell transplants were performed on the patient, he died 18 months after initial presentation.

CONCLUSION:

Due to its rarity, the diagnosis of MCL without typical immunophenotype and genetic aberrations is particularly challenging. Comprehensive investigation of clinical and pathological features to exclude other T+ myeloid neoplasms is necessary.

KEYWORDS:

CD25; KIT; Mast cell leukemia; Systemic mastocytosis; Tryptase

PMID:
29458385
PMCID:
PMC5819157
DOI:
10.1186/s13000-018-0691-2
[Indexed for MEDLINE]
Free PMC Article
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4.
Gastroenterology. 2018 Jul;155(1):23-24.e1. doi: 10.1053/j.gastro.2017.12.029. Epub 2018 Feb 1.

A Case of Cryptogenic Liver Failure.

Author information

1
Division of Gastroenterology, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
2
Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
3
Division of Transplant Surgery, Department of Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
PMID:
29408637
DOI:
10.1053/j.gastro.2017.12.029
[Indexed for MEDLINE]
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5.
Orv Hetil. 2018 Feb;159(5):192-196. doi: 10.1556/650.2018.30978.

[Systemic mastocytosis with progressive disease course].

[Article in Hungarian]

Author information

1
III. Belgyógyászati Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest, Kútvölgyi út 4., 1125.
2
I. Patológiai Intézet, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.
3
Hematológiai Osztály és Patológia Osztály, Jósa András Oktató Kórház Nyíregyháza.
4
Patológiai Intézet, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen.

Abstract

Authors report on a case of a male patient of systemic mastocytosis that was associated with extensive cutaneous lesions. Chronic diarrhoea worsening his quality of life was well managed by the administration of antihistamines. The pleural fluid recurrence soon after drainage has been controlled by the administration of alpha interferon. 40 years after the onset of the first skin signs progression has been manifested in the development of "B" (bone marrow infiltration rate >30%, dysmyelopoiesis, serum tryptase >20 μg/L, hepato- and splenomegaly) and "C" symptoms (liver function test abnormalities, cytopenia, malabsorption, osteoporosis). The patient died at age of 87. The authors' aim was to attract attention on this rare disease and emphasize that symptomatic therapy with antihistamines and drugs available based on customised rights by the National Health Insurance Fund might provide good quality of life. Orv Hetil. 2018; 159(5): 192-196.

KEYWORDS:

chronic diarrhoea; krónikus hasmenés; mastocytosis; mellkasi folyadékgyülem; pleural fluid

PMID:
29376426
DOI:
10.1556/650.2018.30978
[Indexed for MEDLINE]
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6.
Genes Chromosomes Cancer. 2018 May;57(5):252-259. doi: 10.1002/gcc.22526. Epub 2018 Feb 19.

Incidence and prognostic impact of cytogenetic aberrations in patients with systemic mastocytosis.

Author information

1
Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Heidelberg, Germany.
2
Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany.
3
University Hospital Salzburg, Paracelsus Medical University, Salzburg, Austria.
4
Department of Internal Medicine I, Division of Hematology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.
5
MLL Munich Leukemia Laboratory, Munich, Germany.
6
Department of Human Genetics, Hannover Medical School, Hannover, Germany.
7
Faculty of Medicine, University of Southampton, Southampton, United Kingdom.
8
Wessex Regional Genetics Laboratory, Salisbury, United Kingdom.

Abstract

The clinical behavior of systemic mastocytosis (SM) is strongly associated with activating mutations in KIT (D816V in >80% of cases), with the severity of the phenotype influenced by additional somatic mutations, for example, in SRSF2, ASXL1, or RUNX1. Complex molecular profiles are frequently associated with the presence of an associated hematologic neoplasm (AHN) and an unfavorable clinical outcome. However, little is known about the incidence and prognostic impact of cytogenetic aberrations. We analyzed cytogenetic and molecular characteristics of 109 patients (KIT D816V+, n = 102, 94%) with indolent (ISM, n = 26) and advanced SM (n = 83) with (n = 73, 88%) or without AHN. An aberrant karyotype was identified in SM-AHN (16/73, 22%) patients only. In patients with an aberrant karyotype, additional somatic mutations were identified in 12/16 (75%) patients. Seven of 10 (70%) patients with a poor-risk karyotype, for example, monosomy 7 or complex karyotype, and 1/6 (17%) patients with a good-risk karyotype progressed to secondary acute myeloid leukemia (n = 7) or mast cell leukemia (n = 1) within a median of 40 months (range 2-190, P = .04). In advanced SM, the median overall survival (OS) of poor-risk karyotype patients was significantly shorter than in good-risk/normal karyotype patients (4 vs 39 months; hazard ratio 11.7, 95% CI 5.0-27.3; P < .0001). Additionally, the shortened OS in patients with poor-risk karyotype was independent from the mutation status. In summary, a poor-risk karyotype is an independent prognostic variable in advanced SM. Cytogenetic and molecular analyses should be routinely performed in all patients with advanced SM ± AHN because these investigations greatly support prognostication and treatment decisions.

KEYWORDS:

S/A/R panel; cytogenetic aberrations; molecular mutations; systemic mastocytosis

PMID:
29341334
DOI:
10.1002/gcc.22526
[Indexed for MEDLINE]
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8.
J Drugs Dermatol. 2017 Dec 1;16(12):1285-1287.

Mast Cell Burden in a Patient With Cutaneous Disease

Abstract

Mastocytosis is a disease characterized by the abnormal clonal proliferation of mast cells in skin and/or extracutaneous organs, often relating to activating mutations of c-KIT. Histopathology special stains, such as Giemsa, Leder, and Toluidine blue, are key for the diagnosis of cutaneous mastocytosis (CM). In adults, skin lesions can be associated with systemic disease. Tryptase is a diagnostic marker in mastocytosis and thought to reflect the burden of mast cell disease. In this report, we present a case of cutaneous mast cell disease with associated findings of elevated serum tryptase and mast cell infiltration of the bone marrow consistent with indolent systemic mastocytosis.

PMID:
29240865
[Indexed for MEDLINE]
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9.
Vet Comp Oncol. 2018 Mar;16(1):E194-E201. doi: 10.1111/vco.12373. Epub 2017 Dec 14.

Association of prognostic features and treatment on survival time of dogs with systemic mastocytosis: A retrospective analysis of 40 dogs.

Author information

1
College of Veterinary Medicine, Cornell University, Ithaca, New York.
2
Department of Animal Science, College of Agriculture and Life Sciences, Cornell University, Ithaca, New York.
3
Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, New York.
4
Oncology Services, Veterinary Medical Center of Long Island, West Islip, New York.

Abstract

Systemic mastocytosis is a rare phenomenon, with limited information regarding prognostic features and effective treatment of canine patients with this disease. The objective of this study is to determine the impact of certain features and treatments on dogs with systemic mastocytosis. The medical records of 40 dogs from 4 northeastern US veterinary hospitals, with evidence of systemic mast cell disease, were evaluated retrospectively. Variables analysed with relation to overall survival and prognostic significance included treatment protocol used, substage, presence of a cutaneous or visceral tumour, presence of multiple cutaneous Mast cell tumours, grade of the primary tumour and metastatic site(s). Dogs with metastatic disease confined to distant lymph nodes lived longer than those with circulating mast cells in the blood (P = .001), and those with metastatic disease evident in more than 2 sites had a worse prognosis than those with disease in a single location (P = .005). Additionally, administration of chemotherapeutic agents led to increased survival over prednisone therapy alone (P = .008), with the combination of lomustine, vinblastine and prednisone prolonging survival over the tyrosine kinase inhibitor, toceranib phosphate (P = .002). Presence of mast cells in the blood and/or evidence of disease in more than 2 sites indicate widespread dissemination suggesting their use as negative prognostic features. Furthermore, a chemotherapy protocol including combination lomustine and vinblastine therapy may be more effective than toceranib phosphate for the treatment of dogs with disseminated mast cell disease. Overall, patients with systemic mastocytosis have a grave prognosis and more effective treatment options are needed.

KEYWORDS:

chemotherapy; comparative oncology; metastasis; oncology; small animal; tyrosine kinase

PMID:
29239110
DOI:
10.1111/vco.12373
[Indexed for MEDLINE]
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10.
Br J Dermatol. 2017 Nov;177(5):1167-1168. doi: 10.1111/bjd.15962.

Neonatal aggressive systemic mastocytosis.

Author information

1
St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, SE1 7EH, U.K.
2
Department of Dermatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, U.K.
PMID:
29192973
DOI:
10.1111/bjd.15962
[Indexed for MEDLINE]
Icon for Wiley
12.
Gastroenterology. 2018 Mar;154(4):818-819. doi: 10.1053/j.gastro.2017.10.046. Epub 2017 Nov 7.

Portal Hypertension and Unexplained Diarrhea in an 80-Year-Old Man.

Author information

1
Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota.
2
Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota; Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota.
PMID:
29122545
DOI:
10.1053/j.gastro.2017.10.046
[Indexed for MEDLINE]
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13.
Br J Haematol. 2018 Jan;180(1):11-23. doi: 10.1111/bjh.14967. Epub 2017 Oct 19.

How we diagnose and treat systemic mastocytosis in adults.

Author information

1
Department of Hematology and Oncology, Oregon Health and Science University, Portland, OR, USA.
2
Department of Hematology and Oncology, Mayo Clinic, Scottsdale, AZ, USA.

Abstract

Rapid advances in the understanding of the molecular biology, data from translational and clinical trials, and retrospective analyses has influenced the diagnosis and treatment of systemic mastocytosis (SM). Many options have existed for the symptomatic management of SM patients, but recent evolution in regards to the molecular underpinnings of this disease and our ability to distinguish clonal mastocytosis from mast cell activation syndrome has changed our treatment paradigm and opened new opportunities for understanding genetic risk, transformation to mast cell leukaemia, and treatment choices. Key to this change has been the discovery of the KIT mutation and the use of next generation sequencing to evaluate for co-existing molecular mutations that may define the disease course. Careful diagnosis, judicious symptom management and close surveillance of those who may have yet undiagnosed disease is paramount in providing optimal management. In this article, we review the diagnosis and provide a paradigm for the management of SM patients.

KEYWORDS:

imatinib; midostaurin; systemic mastocytosis

PMID:
29048112
DOI:
10.1111/bjh.14967
[Indexed for MEDLINE]
Icon for Wiley
14.
Ann Biol Clin (Paris). 2017 Dec 1;75(6):689-694. doi: 10.1684/abc.2017.1300.

From the observation of atypical cells on blood smear to the diagnosis of mast cell leukemia: a case report in a 79 year old woman consulting for anemia.

Author information

1
Laboratoire d'hématologie, CHR d'Orléans, Orléans, France.
2
Laboratoire d'hématologie, Hôpital de Mercy, CHR Metz-Thionville, France.
3
Service d'hématologie biologique, CHRU de Nancy, France.
4
Service d'hématologie, Hôpital Belle Isle, Hôpitaux privés de Metz, France.

Abstract

Mast cell leukemia is an extremely rare disease, which belongs to the systemic mastocytosis group (WHO 2016). We are reporting the case of a 79-year-old woman, without any hematological particular history consulting for hyperthermia, repeated malaise and subacute anemia. Her clinical examination was normal. Unusual cells were seen on blood and bone marrow smears. They represent more than 10% of blood nucleated cells end more than 20% of the bone marrow nucleated cells. Bone marrow immunophenotyping was performed to characterize these cells. It revealed a cell subset expressing the surface antigens CD117, CD2 and CD25. This immunophenotypic profile is the hallmark of malignant mast cells. Then mast cell leukemia diagnosis could have been made and KIT gene sequencing highlighted the N822Y mutation in exon 17. The patient was initially treated with midostaurin, a tyrosine kinase inhibitor. Lack of therapeutic response and absence of the KIT D816V mutation led to switch to imatinib, following the latest scientific recommendations.

KEYWORDS:

D816V mutation; KIT; atypical mast cells; mast cell leukemia; midostaurin

PMID:
29043985
DOI:
10.1684/abc.2017.1300
[Indexed for MEDLINE]
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16.
Am J Case Rep. 2017 Oct 3;18:1053-1057.

Systemic Mastocytosis in Association with Small Lymphocytic Lymphoma.

Author information

1
Department of Internal Medicine, Southern Illinois University, Springfield, IL, USA.
2
Department of Pathology, Memorial Medical Center, Springfield, IL, USA.
3
Division of Hematology/Oncology, Simmons Cancer Institute at Southern Illinois University (SIU), Springfield, IL, USA.

Abstract

BACKGROUND Systemic mastocytosis with an associated hematologic non-mast cell lineage disease is a rare entity, and the majority of systemic mastocytosis cases are associated with myeloid neoplasm. Lymphoproliferative disorders are less commonly associated with systemic mastocytosis and a few cases of systemic mastocytosis associated with chronic lymphocytic leukemia have been described in the literature. CASE REPORT We present a case of indolent systemic mastocytosis associated with small lymphocytic lymphoma. The bone marrow biopsy demonstrated mast cells in the form of clusters and perivascular distribution on immunohistochemistry for tryptase, CD2, and CD25 markers. In addition, 30% involvement by small lymphocytic lymphoma was discovered in the form of interstitial lymphoid aggregates composed of small lymphocytes. Flow cytometry showed B-cells positively stained for CD19, CD20, CD5, CD23, and kappa light chains, and the CD38 expression was <5%. CONCLUSIONS In systemic mastocytosis with an associated hematologic non-mast cell lineage disease, the combination of systemic mastocytosis associated with small lymphocytic lymphoma is rare and the management strategy follows the principle of treating the two entities individually as if they are not related. Clinical surveillance is indicated for indolent systemic mastocytosis and low-risk small lymphocytic lymphoma to monitor for disease progression.

PMID:
28970467
PMCID:
PMC5637574
[Indexed for MEDLINE]
Free PMC Article
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17.
Immunol Allergy Clin North Am. 2017 Nov;37(4):629-641. doi: 10.1016/j.iac.2017.06.002. Epub 2017 Aug 9.

Drug-Induced Anaphylaxis.

Author information

1
Avenida Eneas de Carvalho Aguiar 155, 8th Floor, Prédio dos Ambulatórios, Bloco 03, 05403-900, Sao Paulo, Brasil. Electronic address: marcelovivoloaun@gmail.com.
2
Avenida Eneas de Carvalho Aguiar 155, 8th Floor, Prédio dos Ambulatórios, Bloco 03, 05403-900, Sao Paulo, Brasil.

Abstract

Drugs are among the main triggers of anaphylaxis, but identification of the culprit drug is frequently difficult. To confirm diagnosis of the causative agent, medical records and clinical history are fundamental. There are a few in vitro tests available in clinical practice, such as serum-specific IgE and basophil activation test. Skin tests are often useful for the diagnosis, although drug challenge is indicated in patients with inconclusive clinical history or to provide safe alternatives. Treatment of anaphylaxis is standard and intramuscular epinephrine is the main agent to prevent morbidity and mortality. Rapid desensitization may be indicated in selected cases.

KEYWORDS:

Adverse drug reactions; Anaphylaxis; Drug allergy; Epinephrine; Hypersensitivity; Nonsteroidal anti-inflammatory drugs

PMID:
28965631
DOI:
10.1016/j.iac.2017.06.002
[Indexed for MEDLINE]
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18.
Expert Rev Clin Pharmacol. 2017 Nov;10(11):1177-1189. doi: 10.1080/17512433.2017.1387051. Epub 2017 Oct 10.

Midostaurin treatment in FLT3-mutated acute myeloid leukemia and systemic mastocytosis.

Author information

1
a Department of Internal Medicine V , University Hospital of Heidelberg , Heidelberg , Germany.
2
b Clinical Cooperation Unit Molecular Hematology/Oncology , German Cancer Research Center (DKFZ) and Department of Internal Medicine V, University of Heidelberg , Heidelberg , Germany.
3
c Sidney Kimmel Comprehensive Cancer Center , Johns Hopkins University , Baltimore , MD , USA.
4
d National Center for Tumor Diseases , Heidelberg , Germany.

Abstract

A number of tyrosine kinase inhibitors (TKIs) have been developed that inhibit the constitutively activated kinase activity caused by activating tyrosine kinase mutations, such as FLT3 or KIT, thus interrupting signaling pathways. Currently, midostaurin is the only approved TKI as monotherapy for aggressive systemic mastocytosis (SM), SM with associated hematological neoplasm, or mast cell leukemia displaying a KIT mutation as well as in combination with standard intensive chemotherapy for adult patients with newly diagnosed FLT3-mutated acute myeloid leukemia (AML). Areas covered: We provide a concise review of the pharmacology, tolerability and clinical efficacy of midostaurin and emerging new treatment options for ASM and FLT3-mutated AML. Expert commentary: Currently, midostaurin is the only approved TKI in aggressive SM, SM with associated hematological neoplasm, or mast cell leukemia inducing responses including complete remissions. With regard to AML, midostaurin is the first drug to receive regulatory approval in this indication in the molecularly defined subgroup of AML with FLT3 mutations. By introduction of this new standard in AML with FLT3 mutations, the bare has been raised for future approvals of next generation FLT3 inhibitors which will be based increasingly on head to head comparisons with midostaurin.

KEYWORDS:

Acute myeloid leukemia; FLT3 mutation; KIT mutation; advanced systemic mastocytosis; clinical trials; efficacy; midostaurin; pharmacology; tolerability; tyrosine kinase inhibitor

PMID:
28960095
DOI:
10.1080/17512433.2017.1387051
[Indexed for MEDLINE]
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19.
Ann Oncol. 2017 Oct 1;28(10):2367-2376. doi: 10.1093/annonc/mdx290.

Midostaurin: a magic bullet that blocks mast cell expansion and activation.

Author information

1
Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.
2
Division of Hematology & Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.
3
Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, USA.
4
Department of Dermatology, University of Luebeck, Luebeck, Germany.
5
Department of Pathology, University of New Mexico, Albuquerque, USA.
6
Institute of Pathology, Paracelsus Medical University Salzburg, Salzburg, Austria.
7
Novartis Oncology, Basel, Switzerland.
8
Centre National de Référence des Mastocytoses, Imagine Institute Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
9
Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
10
LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France.
11
Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany.
12
Department of Hematology and Oncology, University Medical Center Mannheim, Heidelberg University, Mannheim, Germany.
13
Stanford University School of Medicine/Stanford Cancer Institute, Stanford, USA.

Abstract

Clinically relevant features in patients with systemic mastocytosis (SM) include the cosmetic burden of lesional skin, mediator-related symptoms, and organ damage resulting from mast cell (MC) infiltration in advanced forms of SM. Regardless of the SM variant, expansion of neoplastic MC in the skin and other organs is triggered by mutant forms of KIT, the most prevalent being D816V. Activation of MC with subsequent release of chemical mediators is often caused by IgE-dependent mechanisms in these patients. Midostaurin, also known as PKC412, blocks the kinase activity of wild-type KIT and KIT D816V, counteracts KIT-dependent growth of neoplastic MC, and inhibits IgE-dependent mediator secretion. Based on this activity-profile, the drug has been used for treatment of patients with advanced SM. Indeed, encouraging results have been obtained with the drug in a recent multi-center phase II trial in patients with advanced SM, with an overall response rate of 60% and a substantial decrease in the burden of neoplastic MC in various organs. Moreover, midostaurin improved the overall survival and relapse-free survival in patients with advanced SM compared with historical controls. In addition, midostaurin was found to improve mediator-related symptoms and quality of life, suggesting that the drug may also be useful in patients with indolent SM suffering from mediator-related symptoms resistant to conventional therapies or those with MC activation syndromes. Ongoing and future studies will determine the actual value of midostaurin-induced MC depletion and MC deactivation in these additional indications.

KEYWORDS:

IgE; mast cell activation; mast cell leukemia; mast cells; targeted drugs

PMID:
28945834
DOI:
10.1093/annonc/mdx290
[Indexed for MEDLINE]
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20.
Pain Physician. 2017 Sep;20(6):E849-E861.

A Practical Guide for Treatment of Pain in Patients with Systemic Mast Cell Activation Disease.

Author information

1
Abteilung für Anästhesie, Interdisziplinäre Intensivmedizin, Schmerzmedizin/Palli-a-tiv-medizin - Zentrum für Schmerzmedizin, CURA - katholisches Krankenhaus im Sieben-gebirge, Bad Honnef, Germany.
2
Institute of Human Genetics, University Hospital of Bonn, Germany.

Abstract

Systemic mast cell activation disease (MCAD, a subclass of mastocytosis), which has a prevalence of around 17% (at least in the German population), is characterized by accumulation of genetically altered dysfunctional mast cells with abnormal release of these cells' mediators. Since mast cells affect functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing, this disease has to be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity of a generally inflammatory and allergic theme. Pain in its different manifestations is a common symptom in MCAD found in more than three-quarters of the MCAD patients. Because of the specific mast cell-related causes of pain in MCAD it should be treated specifically, if possible, deduced from their putative mast cell mediator-related causes. As yet, there is no official guideline for treatment of MCAD at all. The present review focuses on mast cell mediator-induced acute and chronic pain and the current state of analgesic drug therapy options in MCAD. Due to the high prevalence of MCAD, many physicians are often faced with the issue of pain management in MCAD patients. Hence, our practical guide should contribute to the improvement of patient care.Key words: Pain therapy, mast cell activation disease, mast cell activation syndrome, systemic mastocytosis, mast cell.

PMID:
28934791
[Indexed for MEDLINE]
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