skin (S)
gastrointestinal (GI)
cardiovascular (CV)
neurological (N)
anaphylaxis (A) / Coexistent allergy
(inhalative, nutritive, drug, insect) / Constitutional symptoms / Organ damage / organopathy:
Hepatomegaly (H), Splenomegaly (S),
Adenopathy(A),
Osteopenia/
osteoporosis/
osteolysis(O) / Blood cell count abnormalities / Monitoring period (months) / Status (+: living; -: died) / Treatment
ISM
Case 1, F/48 / - / + / S, GI, CV,A / + / - / - / N / 138 / + / PUVA, H1, H2 blocker
Case 2, M/32 / - / - / - / - / - / A / N / 102 / +
Case 3, M/57 / - / - / - / - / - / - / leucocytosis, eosinophilia / 31 / +
Case 4, M/65 / - / - / - / - / - / - / eosinophilia / 15 / +
Case 5, M/39 / - / + / - / - / - / - / N / 32 / + / PUVA
Case 6, M/38 / - / + / NA / NA / - / - / N / 99 / +
Case 7, M/55 / - / + / NA / NA / - / - / N / 71 / +
Case 8, M/81 / - / - / S / NA / + / H / anaemia, eosinophilia / 13 / +
Case 9, F/44 / - / + / S / + / - / - / N / 30 / + / PUVA
Case 10, F/64 / - / - / A / + / - / O / transient anaemia, leucocytosis, thrombocytosis / 14 / +
Case 11, F/80 / - / - / NA / NA / - / - / N / 13 / +
Case 12, M/31 / - / + / - / - / - / H*, O / N / 79 / + / H1, H2 blocker, PUVA
Case 13, M/66 / - / + / S, GI, N / - / + / O / N / 240 / + / calcium, vitamin D, zoledronic acid, pamidronate,
H1, H2 blocker, dasatinib,
Case 14, F/42 / - / + / S, GI, CV / - / - / H / N / 159 / + / H1,H2 blocker, PUVA, interferon, imatinib
SM-AHNMD
Case 15, M/72 / MDS / - / - / - / - / - / anaemia, thrombocytopenia / 7 / - / red blood cell transfusion
Case 16, M/72 / MDS / - / - / - / - / - / pancytopenia / 27 / - / red blood cell transfusion
Case 17, F/63 / MDS / - / - / - / - / - / anaemia,
thrombocytopenia / 39 / - / red blood cell, platelet transfusion
Case 18, M/70 / AML M1 / - / - / - / - / - / anaemia / 1 / -
Case 19, F/46 / AML M4 2. relapse / - / - / - / - / - / anaemia, thrombocytopenia / 3 / -
Case 20, M/52 / ET / - / - / - / - / - / thrombocytosis / 55 / + / anagrelide
Case 21, F/54 / smouldering MM / - / - / - / - / - / anaemia, thrombocytopenia / 30 / + / observation
Case 22, F/52 / MF / - / - / - / + / H, S / leukocytosis, thrombocytopenia / 13 / - / transfusion
Case 23, F/57 / MF / - / - / - / - / - / anaemia, thrombocytosis, leukocytosis / 23 / + / hydroxyurea, transfusion
Case 24, M/48 / AML / - / - / - / - / H / anaemia,
leukopenia / 6 / - / 7+3, high-dose cytosine arabinoside
Case 25, F/52 / PTCL / - / - / - / - / A (lymphoma) / - / 30 / + / 4 cycles CHOP
Case 26, F/34 / CML / - / - / - / - / - / leukocytosis / 104 / + / interferon, hydroxyurea, imatinib
Case 27, F/43 / iHES / - / - / - / - / H / eosinophilia / 110 / + / steroid, interferon, hydroxyurea, imatinib
Case 28, M/59 / B-ALL / - / - / - / - / - / anaemia, thrombocytopenia / 9 / + / GMALL protocol,
Case 29, M/66 / PV / - / - / - / - / S / polyglobulia / 86 / + / aspirin, hydroxyurea, anagrelide, phlebotomy
ASM
Case 30, F/54 / - / - / S / - / + / H*,S, A / anaemia, thrombocytopenia / 19 / - / imatinib, steroid, interferon, red blood cell, platelet transfusion, 4 cycles vepeside + cytosine arabinoside, DAV
Case 31, F/77 / - / - / - / - / + / H*, O / thrombocytopenia, anaemia / 22 / - / red blood cell transfusion, platelet transfusion, PPI+ steroid
Case 32, M/63 / - / - / - / - / + / H*, S, A / anaemia / 47 / - / interferon, red blood cell transfusion
Case 33, M/59 / - / - / S, N / - / + / H*, A, O / anaemia / 28 / + / interferon
Case 34, M/85 / - / - / - / - / + / H*, S / thrombocytopenia / 2 / - / platelet transfusion
Case 35, M/68 / - / - / - / - / + / H*, O / anaemia / 19 / + / red blood cell transfusion
Supplementum 1.
Main clinical characteristics of each observed patient
Abbreviations:
M: male; F: female; N: no; Y: yes; NA: not available; mediator- related symptoms: S: skin (flush, pruritus); GI: gastrointestinal symptoms (diarrhoea); CV: cardiovascular (palpitation, dizziness, syncope); N: neurologic; A: anaphylaxis
Constitutional symptoms were observed generalized weakness, fatigue, sweats, chills, arthrarlgias, myalgias;
Orga damage/ organopathy: hepatomegaly (H) H*: hepatomegalia with elevated alkaline phosphatase; splenomegaly ( S); Adenopathy (A);Osteopenia/osteoporosis/osteolysis (O);
iHES: idiopathic hypereosinophilic syndrome; MDS: myelodysplastic syndrome; AML: acute myeloid leukaemia; ET: essential thrombocythaemia; MM: multiple myeloma; MF: myelofibrosis; PTCL: peripheral T-cell lymphoma; CML: chronic myeloid leukaemia; B-ALL: B-cell acute lymphoblastic leukaemia; PV: polycythaemia vera; status: living (+), died (-); GMALL: German multicentre acute lymphoblastic leukaemia protocol; CHOP: cyclophosphamide, doxorubicin, vincristine, prednisone