Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome

In the paper

“If an IgE-dependent allergy or other underlying reactive disease (e.g., IgE-dependent or other hypersensitivity disorder) is present in the absence of clonal MC, the diagnosis is secondary MCAS (Table 2, Figure 2).”

What the sources say

• Disputed

  In “Proposed Criteria for the diagnosis of idiopathic mast cell activation syndrome,” the paper calls this a secondary mast cell activation disorder when clonal markers are absent and an allergic or other concurrent disease is established. It proposes an MCAS diagnosis only after secondary diseases associated with mast cell activation have been eliminated, contrary to the cited sentence’s diagnosis of “secondary MCAS.” (LLM, conf=0.84)

  Checked by a reasoning model — may be wrong.

  cites [26] Akin, 2010, Mast cell activation syndrome: proposed diagnostic criteria, J Allergy Clin Immunol sourceExperimental — accuracy not yet measured
• Partly supported

  The Classification of MCAS (pp. 5–6) identifies allergy or another underlying disease causing mast-cell activation as secondary MCAS, rather than primary clonal MCAS. But Table 4 also requires the criteria for mast-cell activation to be fulfilled; the presence of an allergy or reactive disease without clonal mast cells does not, by itself, establish secondary MCAS. (LLM, conf=0.82)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol sourceExperimental — accuracy not yet measured
• Supported

  The Abstract defines secondary MCAS as MCAS with an underlying inflammatory disease, often an IgE-dependent allergy, but no KIT-mutated mast cells. The “Underlying disorders and classification of MCAS” section likewise distinguishes secondary MCAS from primary MCAS with clonal mast cells. (LLM, conf=0.94)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy source

In the paper

“These patients as well as those with cutaneous mastocytosis (CM) or SM are both classified as primary (clonal) MCAS (Table 2).”

What the sources say

• Partly supported

  The Results and discussion and Table 2 identify patients with clonal mast-cell markers but no major systemic-mastocytosis criterion, and separately describe patients with cutaneous or systemic mastocytosis. The paper does not classify these groups as primary (clonal) MCAS. It also reports KIT mutations in only three of the five patients in the first group, rather than establishing that all were KIT-mutated. (LLM, conf=0.88)

  cites [21] Akin, 2007, Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with “idiopathic” anaphylaxis, Blood sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [22] Sonneck, 2007, Diagnostic and subdiagnostic accumulation of mast cells in the bone marrow of patients with anaphylaxis: monoclonal mast cell activation syndrome, Int Arch Allergy Immunol source
• Disputed

  The section on primary mast cell activation identifies patients with one or two minor mastocytosis criteria as having MMAS. But the proposed diagnostic criteria section explicitly says patients with urticaria pigmentosa, a form of cutaneous mastocytosis, are excluded from an MCAS diagnosis; it assigns patients with clonal markers to systemic mastocytosis or MMAS according to WHO criteria. The copy does not classify all these patients together as primary (clonal) MCAS. Page numbers are unavailable in the indexed passages. (LLM, conf=0.86)

  Checked by a reasoning model — may be wrong.

  cites [26] Akin, 2010, Mast cell activation syndrome: proposed diagnostic criteria, J Allergy Clin Immunol sourceExperimental — accuracy not yet measured
• Supported

  The classification table (p. 7) lists CM, SM, and (mono)clonal MCAS as forms of primary MCAS. The diagnostic algorithm (p. 5) identifies patients with only one or two minor SM criteria as having (mono)clonal MCAS; Table 4 (p. 6) specifies that MC activation criteria must also be fulfilled. (LLM, conf=0.90)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol source
• Partly supported

  The ‘Underlying disorders and classification of MCAS’ section classifies MCAS with KIT-mutated, usually CD25-positive clonal mast cells as primary, including cases with CM or SM and cases with neither. Our copy does not specify the claimed threshold of one or two minor SM criteria and no major criterion. (LLM, conf=0.85)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy sourceExperimental — accuracy not yet measured

In the paper

“Based on the underlying condition, patients with MCAS should then be further classified into i) primary MCAS where KIT-mutated, clonal (CD25+) MC are detected (with or without an underlying diagnosis of mastocytosis), ii) secondary MCAS where an underlying non-neoplastic disease, usually an IgE-dependent allergy or other hypersensitivity reaction is detected, and iii) idiopathic MCAS, where no KIT”

What the sources say

• Partly supported

  Split into 3 atoms — 2 confirmed, 1 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol sourceExperimental — accuracy not yet measured
• Supported

  Split into 3 atoms — 3 confirmed, 0 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy source

In the paper

“The likelihood of MCA increases when two or more of such symptoms are documented, and the likelihood is even higher when the symptoms respond to agents blocking mediator effects, mediator production or mediator secretion.”

What the sources say

• Supported

  The Proposed Criteria section requires two or more organ manifestations for a presumptive MCAS diagnosis and treats improvement with drugs targeting mast-cell mediators as further supporting evidence. It cautions that treatment response cannot establish the diagnosis alone; the claim describes increased likelihood, not a definitive diagnosis. Page numbers were not provided in our copy. (LLM, conf=0.90)

  cites [26] Akin, 2010, Mast cell activation syndrome: proposed diagnostic criteria, J Allergy Clin Immunol source
• Partly supported

  The Clinical Symptoms section (p. 3) says the likelihood of systemic MCA increases when signs or symptoms occur in two or more organ systems in parallel, require therapy, and are recurrent or permanent—not merely when two symptoms are documented. The Response to Therapy section (p. 4) supports the second part: a major response to antimediator agents is highly suggestive of MCA, although responses to some agents are nonspecific. (LLM, conf=0.88)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol sourceExperimental — accuracy not yet measured
• Supported

  In “Symptoms of mast cell activation (MCA)” (page not provided), the paper says that two or more recognized symptoms occurring together are suggestive of MCA, and that the likelihood is higher when they respond to drugs blocking mediator effects, production, or release. (LLM, conf=0.94)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy source

In the paper

“Some mediators, like tryptase, are more specific than others for MC and thereby considered as the most precise parameters for the demonstration and documentation of MCA (27,28,37-40).”

What the sources say

• Supported

  In “Increase in Serum Tryptase Levels” (p. 4), the consensus notes that histamine lacks specificity and that histamine and PGD2 have sensitivity limitations. It therefore identifies tryptase as the preferred marker for documenting mast cell activation, which supports the citing sentence’s comparison. (LLM, conf=0.90)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol source
• Supported

  The Laboratory assessment of systemic MCA section says tryptase is more specific for mast cells than other mediators and calls a rapid rise in serum tryptase from baseline a specific, reliable parameter of MCA. It also says other mediators are less specific for MCA. The relevant measure is the increase from baseline, not an elevated tryptase level alone. (LLM, conf=0.91)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy source
• ?Not checked — no full text of the sourcecites [37] Schwartz, 1987, Tryptase levels as an indicator of mast-cell activation in systemic anaphylaxis and mastocytosis, N Engl J Med source
• ?Not checked — no full text of the sourcecites [38] Shanmugam, 2006, Prolonged elevation of serum tryptase in idiopathic anaphylaxis, J Allergy Clin Immunol source
• ?Not checked — no full text of the sourcecites [39] Schwartz, 2006, Diagnostic value of tryptase in anaphylaxis and mastocytosis, Immunol Allergy Clin North Am source
• ?Not checked — no full text of the sourcecites [40] Ferrer, 2010, Serum total tryptase levels are increased in patients with active chronic urticaria, Clin Exp Allergy source

In the paper

“Therefore, in a routine evaluation, a rapid substantial increase in serum tryptase levels above the individual’s baseline is considered specific for MC involvement and MCA, and thus can be employed as a MCAS criterion.”

What the sources say

• Supported

  The “Increase in Serum Tryptase Levels” section (p. 4) says an acute rise of at least 20% plus 2 ng/ml above the individual's baseline is indicative of MCA and meets the MCAS criterion. The same section calls tryptase the preferred marker for documenting MCA, while noting that the proposed threshold still requires further validation. (LLM, conf=0.91)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol source
• Supported

  The “Laboratory assessment of systemic MCA” section calls a rapid rise in serum tryptase from baseline a specific and reliable parameter of MCA. The “Definition of MCA syndromes (MCAS) and related criteria” section includes a tryptase rise as a biochemical criterion for MCAS, preferably using the 20% + 2 ng/ml formula. It is one criterion, not sufficient for diagnosis on its own; page numbers are not provided in our copy. (LLM, conf=0.94)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy source
• ?Not checked — no full text of the sourcecites [37] Schwartz, 1987, Tryptase levels as an indicator of mast-cell activation in systemic anaphylaxis and mastocytosis, N Engl J Med source
• ?Not checked — no full text of the sourcecites [38] Shanmugam, 2006, Prolonged elevation of serum tryptase in idiopathic anaphylaxis, J Allergy Clin Immunol source
• ?Not checked — no full text of the sourcecites [39] Schwartz, 2006, Diagnostic value of tryptase in anaphylaxis and mastocytosis, Immunol Allergy Clin North Am source
• ?Not checked — no full text of the sourcecites [40] Ferrer, 2010, Serum total tryptase levels are increased in patients with active chronic urticaria, Clin Exp Allergy source

In the paper

“However, the sensitivity of the tryptase algorithm decreases with decreasing clinical severity and with delayed blood draws after resolution of clinical symptoms.”

What the sources say

• Supported

  The “Increase in Serum Tryptase Levels” section (p. 4) says tryptase declines after its peak and that event severity affects how long levels remain elevated. It explicitly advises considering both sample timing and clinical severity when interpreting tryptase levels, supporting the paraphrase about reduced sensitivity with milder events and delayed draws. (LLM, conf=0.90)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol source
• Partly supported

  The Laboratory assessment of systemic MCA section says mast-cell mediators are measurable in most severe systemic reactions and describes a rapid rise in tryptase from baseline as a diagnostic parameter. It recommends measuring the baseline after symptoms resolve, but does not state that the algorithm’s sensitivity decreases progressively with milder symptoms or quantify the effect of delayed acute blood draws. The Differential diagnoses section likewise calls for a post-resolution sample to establish baseline, not as a substitute for an acute sample. (LLM, conf=0.77)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [37] Schwartz, 1987, Tryptase levels as an indicator of mast-cell activation in systemic anaphylaxis and mastocytosis, N Engl J Med source
• ?Not checked — no full text of the sourcecites [38] Shanmugam, 2006, Prolonged elevation of serum tryptase in idiopathic anaphylaxis, J Allergy Clin Immunol source
• ?Not checked — no full text of the sourcecites [39] Schwartz, 2006, Diagnostic value of tryptase in anaphylaxis and mastocytosis, Immunol Allergy Clin North Am source
• ?Not checked — no full text of the sourcecites [40] Ferrer, 2010, Serum total tryptase levels are increased in patients with active chronic urticaria, Clin Exp Allergy source

In the paper

“Whenever a major increase in the PGD2 metabolite level is found and the symptoms respond to cyclooxygenase inhibitors, the diagnosis of MCAS may also be considered (27) although the cell source may be ambiguous, particularly if both tryptase and histamine or histamine metabolite levels are normal.”

What the sources say

• Supported

  The citation is attached to the proposal to consider MCAS when a PGD2 metabolite rises substantially and symptoms respond to treatment. The Definition and Proposed MCA Criteria section (p. 3) includes a substantial rise in PGD2 urinary metabolites and a major treatment response among indicators of mast-cell activation; Response to Therapy (p. 4) treats response to cyclooxygenase inhibitors as supportive but nonspecific evidence. The subsequent comment about ambiguity of the cell source is outside the citation’s placement. (LLM, conf=0.88)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol source

In the paper

“hereditary alpha-tryptasemia, an autosomal dominant condition defined by germline replications (usually duplications or triplications) of the TPSAB1 gene encoding alpha-tryptase (64-67).”

What the sources say

• Partly supported

  The “To the Editor” section reports an autosomal dominant inheritance pattern of elevated basal serum tryptase in nine families. It does not identify TPSAB1 duplications or triplications as the cause; the authors say additional genetic study is ongoing. (LLM, conf=0.90)

  cites [64] Lyons, 2014, Mendelian inheritance of elevated serum tryptase associated with atopy and connective tissue abnormalities, J Allergy Clin Immunol sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [65] Lyons, 2016, Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number, Nat Genet source
• Partly supported

  The Introduction (page unavailable) identifies hereditary α-tryptasemia with extra germline copies of α-tryptase-encoding TPSAB1, and the Results (page unavailable) refer to TPSAB1 duplications or triplications. The Results also describe affected families with increased copy number on one allele, but our copy does not explicitly establish autosomal-dominant inheritance or show that duplications and triplications are the usual forms. (LLM, conf=0.81)

  cites [66] Lyons, 2018, A common haplotype containing functional CACNA1H variants is frequently coinherited with increased TPSAB1 copy number, Genet Med sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [67] Sabato, 2018, First identification of an inherited TPSAB1 quintuplication in a patient with clonal mast cell disease, J Clin Immunol source

In the paper

“an elevated basal serum tryptase level per se is neither an indication for MCA or MCAS nor is it an ‘a priori’ risk factor for the occurrence of MCA or MCAS.”

What the sources say

• Partly supported

  The Discussion (p. 7) reports that elevated serum tryptase can come from AML blasts rather than mast cells, supporting the point that an elevated level alone does not establish mast-cell activation. The paper does not assess whether basal tryptase is a risk factor for MCA or MCAS. (LLM, conf=0.84)

  cites [68] Sperr, 2001, Expression of mast cell tryptase by myeloblasts in a group of patients with acute myeloid leukemia, Blood sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [69] Sperr, 2002, Serum tryptase measurements in patients with myelodysplastic syndromes, Leuk Lymphoma source
• ?Not checked — no full text of the sourcecites [70] Sperr, 2009, Elevated tryptase levels selectively cluster in myeloid neoplasms: a novel diagnostic approach and screen marker in clinical haematology, Eur J Clin Invest source
• Partly supported

  The section on the test’s development (page not supplied) identifies a rise above baseline during anaphylaxis—not an elevated basal level—as a diagnostic parameter of mast-cell activation. The screening section also describes elevated basal levels in other conditions. These passages support the distinction between basal elevation and evidence of activation, but do not address whether basal elevation is an a priori risk factor for MCA or MCAS. (LLM, conf=0.79)

  cites [71] Valent, 2014, The serum tryptase test: an emerging robust biomarker in clinical hematology, Expert Rev Hematol sourceExperimental — accuracy not yet measured

In the paper

“If no evidence for primary or secondary MCAS is found, the patient is classified as having idiopathic MCAS (Table 2, Figure 1) (26-28).”

What the sources say

• Partly supported

  The section on proposed criteria for idiopathic MCAS supports ruling out primary and secondary causes before making the diagnosis. It also requires three additional diagnostic criteria; the cited sentence makes exclusion alone sound sufficient for classification. (LLM, conf=0.82)

  cites [26] Akin, 2010, Mast cell activation syndrome: proposed diagnostic criteria, J Allergy Clin Immunol sourceExperimental — accuracy not yet measured
• Supported

  The Classification of MCAS (pp. 5–6) defines idiopathic MCAS as meeting mast-cell activation criteria without identified mast-cell clonality or an underlying cause. Its Table 4 adds that this is a final diagnosis requiring an extensive workup to exclude other conditions. (LLM, conf=0.93)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol sourceExperimental — accuracy not yet measured
• Supported

  The 'Underlying disorders and classification of MCAS' section says that a patient already diagnosed with MCAS should be classified as having idiopathic MCAS when neither a secondary cause, such as allergy or another inflammatory process, nor evidence of a clonal mast-cell disorder is detectable. This supports the citing sentence's classification rule. (LLM, conf=0.94)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy sourceExperimental — accuracy not yet measured

In the paper

“an elevated basal tryptase level is not only found in patients with mastocytosis or hereditary alpha-tryptasemia (where the risk for anaphylaxis may be increased) but also in patients with myeloid non-MC-lineage neoplasms (Supplemental Table S1) (68-71).”

What the sources say

• Partly supported

  The Discussion (p. 7) identifies tryptase as a marker of mastocytosis, and the Results (p. 3) report elevated serum tryptase in subsets of patients with acute myeloid leukemia, a non-mast-cell-lineage neoplasm. The paper does not address hereditary alpha-tryptasemia or its possible association with anaphylaxis risk. (LLM, conf=0.88)

  cites [68] Sperr, 2001, Expression of mast cell tryptase by myeloblasts in a group of patients with acute myeloid leukemia, Blood sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [69] Sperr, 2002, Serum tryptase measurements in patients with myelodysplastic syndromes, Leuk Lymphoma source
• ?Not checked — no full text of the sourcecites [70] Sperr, 2009, Elevated tryptase levels selectively cluster in myeloid neoplasms: a novel diagnostic approach and screen marker in clinical haematology, Eur J Clin Invest source
• Partly supported

  The sections on elevated basal serum tryptase and cellular origin report elevated levels in systemic mastocytosis and several other myeloid neoplasms, including AML and CML (page not provided). The paper also mentions familial mast-cell activation syndromes, but the passages do not identify hereditary alpha-tryptasemia or link that condition to increased anaphylaxis risk. Its discussion of anaphylaxis concerns a rise in tryptase during a reaction, not that risk claim. (LLM, conf=0.87)

  cites [71] Valent, 2014, The serum tryptase test: an emerging robust biomarker in clinical hematology, Expert Rev Hematol sourceExperimental — accuracy not yet measured

In the paper

“The induction of long-term tolerance to an insect venom allergen is reduced in primary MCAS and severe or even fatal reactions after discontinuation of immunotherapy have been described (71,72).”

What the sources say

• ?Could not verify

  The 'Value of tryptase testing in patients with mastocytosis' section (page unavailable) discusses hymenoptera venom allergy as a context for screening for systemic mastocytosis, not as an immunotherapy outcome. Searches for long-term venom tolerance and reactions after discontinuing immunotherapy found no passage in our copy addressing either claim. (LLM, conf=0.74)

  cites [71] Valent, 2014, The serum tryptase test: an emerging robust biomarker in clinical hematology, Expert Rev Hematol sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [72] Oude Elberink, 1997, Fatal anaphylaxis after a yellow jacket sting, despite venom immunotherapy, in two patients with mastocytosis, J Allergy Clin Immunol source

In the paper

“The consensus group has recommended that in cases with mastocytosis (irrespective of the variant) any form of MCA requiring continuous mediator-targeted therapy should be marked by the diagnostic label ‘SY’”

What the sources say

• Partly supported

  The Mastocytosis section (p. 7) says MCA can occur in any variant of mastocytosis and that its presence may be recorded with the subscript ‘SY’. The Response to Therapy section (p. 4) describes SM SY as mediator-related symptoms requiring therapy, but does not specify continuous therapy or explicitly recommend SY for cases that do not fulfill MCAS criteria. (LLM, conf=0.84)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol sourceExperimental — accuracy not yet measured
• ?Not checked — no full text of the sourcecites [63] Valent, 2007, Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria, Eur J Clin Invest source

In the paper

“Diagnostic MCAS criteria include typical clinical symptoms, often with hypotension, an event-related, substantial increase in serum tryptase levels and response of clinical symptoms to MC-stabilizing drugs or drugs counteracting the effects of MC-derived mediators.”

What the sources say

• Supported

  The Definition and Proposed MCA Criteria section (p. 3, table 2) lists typical symptoms, an event-related serum tryptase increase, and symptom response to histamine blockers or MC-targeting agents. The same section lists hypotension among typical symptoms; the Response to Therapy section (p. 4) also discusses drugs that counteract MC mediators. (LLM, conf=0.94)

  cites [27] Valent, 2012, Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol source
• Supported

  The ‘Definition of MCA syndromes (MCAS) and related criteria’ section lists clinical signs, a biochemical increase in tryptase, and response to mast-cell-stabilizing or mediator-directed therapy as the three diagnostic criteria. The ‘Symptoms of mast cell activation (MCA)’ section identifies hypotension or shock as a key finding. Page numbers were not supplied in our copy. (LLM, conf=0.94)

  cites [28] Valent, 2013, Mast cell activation syndromes: definition and classification, Allergy source

In the paper

“In affected family members, symptoms of MCA, if present, may be chronic and/or acute, and other symptoms and findings, including dysautonomia, chronic pain, and connective tissue abnormalities such as joint hypermobility, may also be observed (64-67).”

What the sources say

• Supported

  The 'To the Editor' section reports chronic and episodic symptoms consistent with mast cell degranulation in affected family members. It also reports autonomic dysfunction, chronic musculoskeletal pain, and a hypermobile connective tissue phenotype. Page numbers were not provided in our copy. (LLM, conf=0.93)

  cites [64] Lyons, 2014, Mendelian inheritance of elevated serum tryptase associated with atopy and connective tissue abnormalities, J Allergy Clin Immunol source
• ?Not checked — no full text of the sourcecites [65] Lyons, 2016, Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number, Nat Genet source
• Supported

  Supplementary Table 2 (page unavailable) reports anaphylaxis and flushing/pruritus alongside chronic reflux symptoms in individuals with TPSAB1 duplications. It also records autonomic symptom scores, body pain/headache, and joint hypermobility. These findings support the cited sentence’s qualified description of symptoms and findings that may occur in affected family members. (LLM, conf=0.88)

  cites [66] Lyons, 2018, A common haplotype containing functional CACNA1H variants is frequently coinherited with increased TPSAB1 copy number, Genet Med source
• ?Not checked — no full text of the sourcecites [67] Sabato, 2018, First identification of an inherited TPSAB1 quintuplication in a patient with clonal mast cell disease, J Clin Immunol source

In the paper

“The prevalence of MCAS in pediatric patients is unknown, but both cutaneous and systemic reactions have been reported (82).”

What the sources say

• ?Not checked — no full text of the sourcecites [82] Matito, 2015, Cutaneous and systemic mastocytosis in children: a risk factor for anaphylaxis?, Curr Allergy Asthma Rep source

In the paper

“This approach has recently been validated in the context of mastocytosis (PV, KB and LBS, unpublished data) and non-mastocytosis conditions (44).”

What the sources say

• ?Not checked — no full text of the sourcecites [44] Baretto, 2017, Validation of international consensus equation for acute serum total tryptase in mast cell activation: a perioperative perspective, Allergy source

In the paper

“Indirect signs for the presence of an underlying (occult) SM include an elevated baseline serum tryptase level detected well after complete resolution of all symptoms or a D816V KIT mutation in circulating blood leukocytes, blood count abnormalities (e.g., eosinophilia), symptoms suggestive of SM such as unexplained osteoporosis, gastrointestinal symptoms (diarrhea, abdominal cramps, malabsorption”

What the sources say

• ?Not checked — no full text of the sourcecites [60] Alvarez-Twose, 2012, Validation of the REMA score for predicting mast cell clonality and systemic mastocytosis in patients with systemic mast cell activation symptoms, Int Arch Allergy Immunol source

In the paper

“The diagnosis of anaphylaxis does not require these MCAS criteria.”

In the paper

“In these cases, a provisional diagnosis of “possibly MCAS” may be established and further treatment should be introduced.”

In the paper

“Rather, MC typing is recommended as a diagnostic approach to diagnose or exclude SM in patients with MCA or MCAS (56-58).”

What the sources say

• ?Not checked — no full text of the sourcecites [56] Alvarez-Twose, 2010, Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms, J Allergy Clin Immunol source
• ?Not checked — no full text of the sourcecites [57] Alvarez-Twose, 2014, Nonaggressive systemic mastocytosis (SM) without skin lesions associated with insect-induced anaphylaxis shows unique features versus other indolent SM, J Allergy Clin Immunol source
• ?Not checked — no full text of the sourcecites [58] Matito, 2014, Anaphylaxis as a clinical manifestation of clonal mast cell disorders, Curr Allergy Asthma Rep source

In the paper

“MCAS is a well-defined condition that occurs primarily in patients with IgE-dependent allergies and/or mastocytosis, but may also occur in a number of other conditions.”

In the paper

“A diagnosis of MCAS is further supported by demonstrating an event-related increase in tryptase and a sustained prophylactic response to MC-stabilizing drugs or drugs directed against MC mediators.”

In the paper

“Therefore, MC typing is not recommended as a screening approach to define or quantify MCA.”

In the paper

“the response of the symptoms to MC-stabilizing and/or anti-mediator-type drugs confirms the presence of MCAS.”