The Pulmonary Manifestation of Mastocytosis: Experiences of the National Reference Centre of Excellence
Abstract
1. Introduction
Aim
2. Materials and Methods
2.1. Inclusion and Exclusion Criteria
2.2. Study Design
2.3. Systemic and Cutaneous Mastocytosis–Diagnostic Criteria
2.4. Evaluation of the Respiratory Function
2.5. Statistical Analysis
3. Results
3.1. Patients with Mastocytosis in Comparison to Healthy Controls
3.2. Clinical Manifestation of Mastocytosis
3.3. Clinical Manifestations of Pulmonary Diseases and Assessment of Respiratory Function
3.4. Impairment of CO Diffusion
4. Discussion
4.1. Respiratory Symptoms
4.2. Dysfunction of the Diffusing Capacity for Carbon Monoxide
4.3. Obstruction
4.4. Limitations
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Variable | Mastocytosis | Controls | p-Value |
---|---|---|---|
Count | 104 (59.4) | 71 (40.6) | - |
Male (%) | 36.0 (34.6) | 36.0 (50.7) | 0.049 |
Age (years) | 46.0 (44.1–49.0) | 39.0 (38.2–45.0) | 0.017 |
Height (cm) | 169.0 (166.2–170.7) | 170.0 (168.3–172.5) | 0.416 |
Body mass (kg) | 75.0 (73.3–80.9) | 79.0 (76.3–86.1) | 0.153 |
BMI (kg/m2) | 25.7 (25.7–27.4) | 25.8 (26.3–29.4) | 0.495 |
Smoking history | 44.0 (42.3) | 0.0 (0.0) | <0.001 |
Multivariate linear regression | |||
FEV1 (l) | 3.1 (3.1–3.4) | 3.5 (3.2–3.6) | 0.054 |
FEV1-predicted value | 1.0 (1.0–1.1) | 1.0 (0.987–1.0) | 0.151 |
FEV1/VC MAX % | 73.7 (71.8–75.0) | 79.4 (76.9–79.8) | <0.001 |
DLCO/VA (mL/min/mmHg/L) | 1.4 (1.4–1.5) | 6.0 (5.9–6.5) | <0.001 |
DLCO/VA-predicted value | 0.895 (0.876–0.926) | 1.003 (0.986–1.009) | <0.001 |
DLCOcSB (mL/min/mmHg) | 7.6 (7.5–8.2) | 9.5 (9.2–10.1) | <0.001 |
DLCOcSB-predicted value | 0.831 (0.824–0.876) | 0.994 (0.982–1.030) | <0.001 |
Variable | PISM | ISM | BMM | MPCM | MCL/ASM | SSM | p-Value |
---|---|---|---|---|---|---|---|
Count | 9 (8.7) | 65 (62.5) | 9 (8.7) | 13 (12.5) | 7 (6.7) | 1 (1.0) | - |
Males (%) | 3.0 (33.3) | 22.0 (33.8) | 6.0 (66.7) | 3.0 (23.1) | 2.0 (28.6) | 0.0 (0.0) | 0.356 |
Age | 48.0 (42.9–59.8) | 43.0 (40.9–46.7) | 58.0 (46.4–62.9) | 42.0 (35.6–56.8) | 58.0 (47.9–63.8) | 49.0 (49.0–49.0) | 0.021 |
Height | 160.0 (155.8–172.6) | 169.0 (165.6–171.8) | 172.0 (163.0–179.4) | 169.0 (165.0–172.4) | 168.0 (155.4–178.9) | 169.0 (169.0–169.0) | 0.765 |
Weight | 69.0 (59.5–92.5) | 72.0 (71.6–82.5) | 83.0 (79.6–94.6) | 76.0 (69.1–80.5) | 64.0 (57.6–83.2) | 80.0 (80.0–80.0) | 0.224 |
BMI | 28.4 (24.1–31.2) | 25.1 (24.9–27.2) | 29.4 (26.1–33.9) | 26.3 (24.5–28.0) | 25.5 (22.5–28.0) | 28.0 (28.0–28.0) | 0.223 |
Age-first symptoms | 38.0 (33.1–43.8) | 30.0 (26.7–32.0) | 48.0 (40.0–56.2) | 31.0 (21.6–43.0) | 42.0 (31.7–51.7) | 42.0 (42.0–42.0) | <0.001 |
Serum tryptase concentration | 17.7 (12.6–26.1) | 32.7 (36.7–56.7) | 30.4 (24.4–53.9) | 12.9 (9.4–17.0) | 71.5 (14.1–271.0) | 206.0 (206.0–206.0) | <0.001 |
Bone marrow CD2: + | 3.0 (33.3) | 53.0 (81.5) | 7.0 (77.8) | 5.0 (38.5) | 4.0 (57.1) | 0.0 (0.0) | <0.001 |
Bone marrow CD25: + | 4.0 (44.4) | 56.0 (86.2) | 9.0 (100.0) | 6.0 (46.2) | 5.0 (71.4) | 1.0 (100.0) | <0.001 |
Bone marrow histopathology mast cells: sparse | 9.0 (100.0) | 9.0 (13.8) | 1.0 (11.1) | 13.0 (100.0) | 2.0 (28.6) | 0.0 (0.0) | <0.001 |
Bone marrow histopathology mast cells: many | 0.0 (0.0) | 56.0 (86.2) | 8.0 (88.9) | 0.0 (0.0) | 5.0 (71.4) | 1.0 (100.0) | |
Spindle-shaped mast cells in bone marrow: 0–1% | 8.0 (88.9) | 30.0 (46.2) | 7.0 (77.8) | 11.0 (84.6) | 2.0 (28.6) | 0.0 (0.0) | 0.003 |
Spindle-shaped mast cells in bone marrow: 1–5 | 1.0 (11.1) | 25.0 (38.5) | 1.0 (11.1) | 2.0 (15.4) | 3.0 (42.9) | 0.0 (0.0) | |
Spindle-shaped mast cells in bone marrow: 5–10 | 0.0 (0.0) | 7.0 (10.8) | 0.0 (0.0) | 0.0 (0.0) | 1.0 (14.3) | 0.0 (0.0) | |
Spindle-shaped mast cells in bone marrow: >10 | 0.0 (0.0) | 3.0 (4.6) | 1.0 (11.1) | 0.0 (0.0) | 1.0 (14.3) | 1.0 (100.0) | |
Bone marrow D816V mutation | 9.0 (100.0) | 50.0 (76.9) | 5.0 (55.6) | 0.0 (0.0) | 6.0 (85.7) | 0.0 (0.0) | 0.002 |
Major criterion | 0.0 (0.0) | 56.0 (86.2) | 8.0 (88.9) | 0.0 (0.0) | 5.0 (71.4) | 1.0 (100.0) | <0.001 |
Minor criterion: atypical morphology | 0.0 (0.0) | 1.0 (1.5) | 1.0 (11.1) | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 0.458 |
Minor criterion: CD2/CD25 expression | 4.0 (44.4) | 58.0 (89.2) | 9.0 (100.0) | 6.0 (46.2) | 5.0 (71.4) | 1.0 (100.0) | <0.001 |
Minor criterion: KIT mutation (D816V) | 9.0 (100.0) | 50.0 (76.9) | 5.0 (55.6) | 0.0 (0.0) | 6.0 (85.7) | 0.0 (0.0) | <0.001 |
Minor criterion: elevated serum tryptase | 2.0 (22.2) | 46.0 (70.8) | 8.0 (88.9) | 2.0 (15.4) | 7.0 (100.0) | 1.0 (100.0) | <0.001 |
Overall SM criteria fulfilment | 0.0 (0.0) | 65.0 (100.0) | 9.0 (100.0) | 0.0 (0.0) | 7.0 (100.0) | 1.0 (100.0) | <0.001 |
Skin involvement | 9.0 (100.0) | 64.0 (98.5) | 0.0 (0.0) | 13.0 (100.0) | 5.0 (71.4) | 1.0 (100.0) | <0.001 |
Percentage area of skin involvement | 65.0 (49.2–90.8) | 81.0 (57.0–73.1) | 0.0 (0.0–0.0) | 90.0 (62.5–89.5) | 45.0 (4.2–68.4) | 90.0 (90.0–90.0) | <0.001 |
Darier’s sign | 7.0 (77.8) | 53.0 (81.5) | 0.0 (0.0) | 12.0 (92.3) | 4.0 (57.1) | 1.0 (100.0) | <0.001 |
Splenomegaly | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 3.0 (42.9) | 0.0 (0.0) | <0.001 |
Hepatomegaly | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 2.0 (28.6) | 1.0 (100.0) | <0.001 |
Lymphadenopathy | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 1.0 (14.3) | 0.0 (0.0) | 0.006 |
Osteoporosis | 1.0 (11.1) | 7.0 (10.8) | 1.0 (11.1) | 1.0 (7.7) | 3.0 (42.9) | 0.0 (0.0) | 0.300 |
Osteopenia | 1.0 (11.1) | 16.0 (24.6) | 1.0 (11.1) | 3.0 (23.1) | 3.0 (42.9) | 0.0 (0.0) | 0.658 |
Bone pain | 5.0 (55.6) | 15.0 (23.1) | 4.0 (44.4) | 2.0 (15.4) | 4.0 (57.1) | 0.0 (0.0) | 0.900 |
Flushing | 5.0 (55.6) | 31.0 (47.7) | 0.0 (0.0) | 6.0 (46.2) | 5.0 (71.4) | 1.0 (100.0) | 0.050 |
Itching | 8.0 (88.9) | 52.0 (80.0) | 0.0 (0.0) | 12.0 (92.3) | 7.0 (100.0) | 1.0 (100.0) | 0.000 |
Angioedema | 4.0 (44.4) | 9.0 (13.8) | 0.0 (0.0) | 3.0 (23.1) | 0.0 (0.0) | 0.0 (0.0) | 0.080 |
Recurring hypotension | 4.0 (44.4) | 14.0 (21.5) | 0.0 (0.0) | 4.0 (30.8) | 3.0 (42.9) | 0.0 (0.0) | 0.323 |
Anaphylaxis | 3.0 (33.3) | 22.0 (33.8) | 9.0 (100.0) | 4.0 (30.8) | 5.0 (71.4) | 0.0 (0.0) | 0.002 |
Diarrhea | 2.0 (22.2) | 13.0 (20.0) | 1.0 (11.1) | 3.0 (23.1) | 4.0 (57.1) | 0.0 (0.0) | 0.322 |
Stomach pain | 2.0 (22.2) | 17.0 (26.2) | 0.0 (0.0) | 3.0 (23.1) | 5.0 (71.4) | 0.0 (0.0) | 0.048 |
Headache | 3.0 (33.3) | 12.0 (18.5) | 4.0 (44.4) | 6.0 (46.2) | 1.0 (14.3) | 0.0 (0.0) | 0.133 |
Allergy | 3.0 (33.3) | 25.0 (38.5) | 7.0 (77.8) | 2.0 (15.4) | 6.0 (85.7) | 0.0 (0.0) | 0.009 |
Food allergy | 2.0 (22.2) | 4.0 (6.2) | 1.0 (11.1) | 0.0 (0.0) | 3.0 (42.9) | 0.0 (0.0) | 0.024 |
Hymenoptera allergy | 9.0 (100.0) | 49.0 (75.4) | 2.0 (22.2) | 11.0 (84.6) | 7.0 (100.0) | 1.0 (100.0) | 0.001 |
Drug allergy | 3.0 (33.3) | 12.0 (18.5) | 2.0 (22.2) | 2.0 (15.4) | 2.0 (28.6) | 0.0 (0.0) | 0.869 |
B-findings | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 3.0 (42.9) | 1.0 (100.0) | <0.001 |
C-findings | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 7.0 (100.0) | 0.0 (0.0) | <0.001 |
Variable | PISM | ISM | BMM | MPCM | MCL/ASM | SSM | p-Value |
---|---|---|---|---|---|---|---|
Dyspnea | 4.0 (44.4) | 23.0 (35.4) | 1.0 (11.1) | 5.0 (38.5) | 5.0 (71.4) | 0.0 (0.0) | 0.934 |
Cough | 1.0 (11.1) | 6.0 (9.2) | 0.0 (0.0) | 1.0 (7.7) | 0.0 (0.0) | 0.0 (0.0) | 0.820 |
Wheezing | 0.0 (0.0) | 7.0 (10.8) | 0.0 (0.0) | 3.0 (23.1) | 0.0 (0.0) | 0.0 (0.0) | 0.350 |
Asthma | 0.0 (0.0) | 6.0 (9.2) | 0.0 (0.0) | 1.0 (7.7) | 1.0 (14.3) | 0.0 (0.0) | 0.817 |
Airway obstruction | 0.0 (0.0) | 7.0 (10.8) | 0.0 (0.0) | 1.0 (7.7) | 0.0 (0.0) | 0.0 (0.0) | 0.654 |
Has ever smoked | 3.0 (33.3) | 26.0 (40.0) | 4.0 (44.4) | 8.0 (61.5) | 2.0 (28.6) | 1.0 (100.0) | 0.503 |
FEV1 | 2.8 (2.5–3.6) | 3.2 (3.1–3.5) | 3.2 (2.5–3.9) | 3.0 (2.6–3.6) | 3.0 (1.9–4.2) | 3.2 (3.2–3.2) | 0.721 |
FEV1 relative to predicted value | 1.1 (0.988–1.3) | 1.0 (1.0–1.1) | 0.948 (0.927–1.1) | 1.0 (0.955–1.1) | 1.1 (0.875–1.3) | 1.1 (1.1–1.1) | 0.463 |
FEV1/FVC | 81.6 (74.4–85.2) | 81.8 (78.7–81.9) | 76.1 (72.1–80.7) | 81.3 (75.0–83.4) | 81.8 (76.3–86.4) | 89.8 (89.8–89.8) | 0.351 |
FEV1/VC MAX | 80.6 (72.6–83.4) | 79.8 (76.7–80.2) | 72.5 (70.8–79.0) | 77.6 (73.2–81.5) | 79.8 (71.8–93.7) | 88.9 (88.9–88.9) | 0.251 |
TLC | 5.6 (4.6–6.3) | 5.6 (5.3–5.9) | 6.3 (5.1–6.8) | 5.5 (4.9–5.9) | 5.0 (3.5–7.2) | 5.0 (5.0–5.0) | 0.891 |
TLC relative to predicted value | 1.0 (0.926–1.1) | 0.972 (0.952–1.0) | 1.0 (0.899–1.0) | 0.982 (0.919–1.0) | 0.982 (0.803–1.1) | 0.935 (0.935–0.935) | 0.798 |
RV | 1.8 (1.1–2.4) | 1.6 (1.4–3.4) | 1.8 (1.5–2.1) | 1.5 (1.4–1.7) | 1.6 (−6.534–21.2) | 1.2 (1.2–1.2) | 0.629 |
RV relative to predicted value | 0.803 (0.714–1.0) | 0.849 (0.834–0.917) | 0.829 (0.755–0.946) | 0.848 (0.792–0.894) | 0.918 (0.785–1.0) | 0.679 (0.679–0.679) | 0.793 |
DLCOcSB | 7.3 (5.9 - 9.2) | 7.7 (7.6–8.5) | 8.5 (7.2–9.4) | 7.5 (6.7–8.8) | 5.8 (4.5–9.0) | 6.8 (6.8–6.8) | 0.501 |
DLCOcSB relative to predicted value | 0.857 (0.752–1.0) | 0.817 (0.814–0.879) | 0.944 (0.812–1.0) | 0.858 (0.786–0.915) | 0.734 (0.622–0.942) | 0.777 (0.777–0.777) | 0.458 |
DLCO/VA | 1.4 (1.3–1.5) | 1.4 (1.4–1.5) | 1.4 (1.3–1.7) | 1.6 (1.3–1.7) | 1.3 (1.1–1.4) | 1.4 (1.4–1.4) | 0.321 |
DLCO/VA relative to predicted value | 0.891 (0.761–1.0) | 0.874 (0.864–0.927) | 1.0 (0.914–1.0) | 0.917 (0.858–0.989) | 0.809 (0.689–0.988) | 0.89 (0.89–0.89) | 0.326 |
MEF 75 (%) | 1.0 (0.922–1.3) | 1.0 (0.943–1.1) | 0.912 (0.796–1.2) | 0.994 (0.834–1.1) | 1.1 (0.913–1.1) | 1.3 (1.3–1.3) | 0.550 |
MEF 50 (%) | 0.927 (0.66–1.1) | 0.85 (0.824–0.954) | 0.784 (0.594–1.0) | 0.849 (0.688–0.995) | 0.972 (0.643–1.2) | 1.5 (1.5–1.5) | 0.260 |
MEF 25 (%) | 0.683 (0.436–0.838) | 0.631 (0.594–0.719) | 0.451 (0.307–0.73) | 0.581 (0.455–0.732) | 0.891 (0.396–1.2) | 1.3 (1.3–1.3) | 0.089 |
MEF 75/25 (%) | 0.913 (0.611–1.0) | 0.822 (0.754–0.875) | 0.643 (0.493–0.901) | 0.764 (0.611–0.87) | 0.852 (0.53–1.1) | 1.4 (1.4–1.4) | 0.112 |
Chest pain—never | 7.0 (77.8) | 53.0 (81.5) | 6.0 (66.7) | 9.0 (69.2) | 5.0 (71.4) | 1.0 (100.0) | 0.601 |
Chest pain—less than once a month | 0.0 (0.0) | 5.0 (7.7) | 1.0 (11.1) | 0.0 (0.0) | 2.0 (28.6) | 0.0 (0.0) | |
Chest pain—every month | 1.0 (11.1) | 6.0 (9.2) | 1.0 (11.1) | 2.0 (15.4) | 0.0 (0.0) | 0.0 (0.0) | |
Chest pain—every week | 1.0 (11.1) | 0.0 (0.0) | 1.0 (11.1) | 1.0 (7.7) | 0.0 (0.0) | 0.0 (0.0) | |
Chest pain—every day | 0.0 (0.0) | 1.0 (1.5) | 0.0 (0.0) | 1.0 (7.7) | 0.0 (0.0) | 0.0 (0.0) |
Variable | Normal CO Diffusion | Impaired CO Diffusion | p-Value |
---|---|---|---|
Count | 99 (95.2) | 5 (4.8) | - |
FEV1 | 3.1 (3.1–3.4) | 3.3 (3.0–3.9) | 0.258 |
FEV1 relative to predicted value | 1.0 (1.0–1.1) | 1.1 (0.997–1.2) | 0.444 |
FEV1/FVC | 81.2 (78.6–81.2) | 83.6 (69.9–91.8) | 0.698 |
FEV1/VC MAX | 79.2 (76.8–79.7) | 83.5 (67.7–91.0) | 0.514 |
Airway obturation | 7.0 (7.1) | 1.0 (20.0) | 0.843 |
TLC | 5.5 (5.3–5.8) | 5.9 (5.1–6.5) | 0.667 |
TLC relative to predicted value | 0.972 (0.951–0.996) | 1.1 (0.985–1.2) | 0.063 |
RV | 1.6 (1.5–3.6) | 1.6 (1.4–1.7) | 0.790 |
RV relative to predicted value | 0.844 (0.834–0.897) | 0.918 (0.879–0.968) | 0.158 |
DLCOcSB | 7.7 (7.6–8.3) | 6.6 (5.3–8.0) | 0.135 |
DLCOcSB relative to predicted value | 0.839 (0.831–0.883) | 0.73 (0.603–0.822) | 0.017 |
DLCO/VA | 1.4 (1.4–1.5) | 1.1 (1.0–1.2) | 0.001 |
DLCO/VA relative to predicted value | 0.902 (0.889–0.936) | 0.656 (0.625–0.703) | <0.001 |
MEF 75 (%) | 1.0 (0.961–1.0) | 1.1 (0.697–1.3) | 0.908 |
MEF 50 (%) | 0.85 (0.829–0.938) | 0.972 (0.556–1.2) | 0.923 |
MEF 25 (%) | 0.625 (0.589–0.698) | 0.891 (0.427–1.1) | 0.207 |
MEF 75/25 (%) | 0.82 (0.748–0.85) | 0.954 (0.536–1.2) | 0.622 |
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Sztormowska, M.; Górska, A.; Piskunowicz, M.; Górska, L.; Nazar, W.; Chełmińska, M.; Kuziemski, K.; Jassem, E.; Niedoszytko, M. The Pulmonary Manifestation of Mastocytosis: Experiences of the National Reference Centre of Excellence. J. Clin. Med. 2025, 14, 5455. https://doi.org/10.3390/jcm14155455
Sztormowska M, Górska A, Piskunowicz M, Górska L, Nazar W, Chełmińska M, Kuziemski K, Jassem E, Niedoszytko M. The Pulmonary Manifestation of Mastocytosis: Experiences of the National Reference Centre of Excellence. Journal of Clinical Medicine. 2025; 14(15):5455. https://doi.org/10.3390/jcm14155455
Chicago/Turabian StyleSztormowska, Marlena, Aleksandra Górska, Maciej Piskunowicz, Lucyna Górska, Wojciech Nazar, Marta Chełmińska, Krzysztof Kuziemski, Ewa Jassem, and Marek Niedoszytko. 2025. "The Pulmonary Manifestation of Mastocytosis: Experiences of the National Reference Centre of Excellence" Journal of Clinical Medicine 14, no. 15: 5455. https://doi.org/10.3390/jcm14155455
APA StyleSztormowska, M., Górska, A., Piskunowicz, M., Górska, L., Nazar, W., Chełmińska, M., Kuziemski, K., Jassem, E., & Niedoszytko, M. (2025). The Pulmonary Manifestation of Mastocytosis: Experiences of the National Reference Centre of Excellence. Journal of Clinical Medicine, 14(15), 5455. https://doi.org/10.3390/jcm14155455