In 50% of people with Sweet’s syndrome (SS) something triggers their condition, but in the remaining 50% there is no known trigger.
In 15-20% of cases, SS can develop secondary to blood cancer and other forms of cancer (Chen et al, 2016). This is called malignancy-associated Sweet’s syndrome (MASS). Myelodysplasia, otherwise known as myelodysplastic syndromes (MDS), is one of the most common causes of MASS.
In all SS patients, cancer needs to be ruled out as a trigger, and you should be checked for MDS via blood tests for at least 6 months after diagnosis. These tests by themselves can’t be used to accurately diagnose MDS, but may indicate it. Unfortunately, SS patients aren’t always being given regular blood tests, so if you have SS you might want to check with your doctor that this is going to be done or has been done. If you have suspected MDS then you’ll need a bone marrow biopsy to confirm diagnosis and determine what kind of MDS you have.
Read more about triggers for SS here.
What is myelodysplastic syndromes?
MDS is a group of blood disorders in which the bone marrow produces too few mature and/or functioning red blood cells, white blood cells or platelets (John Hopkins, 2018). It begins with a change to a normal stem cell in the bone marrow, a stem cell being a cell that has the potential to develop into many different cell types. Sometimes, MDS can progress to a rare type of cancer called acute myeloid leukaemia (AML). In the UK, around 2,600 people are diagnosed with AML each year, and it’s most common in people over 65 (NHS Choices, 2016). MASS only occurs in 1% of AML patients (Guarneri et al, 2018).
See ‘Further information’ and ‘References’ to learn more about MDS and AML, particularly the orange highlighted links.
Myelodysplasia and Sweet’s syndrome.
Is malignancy-associated Sweet’s syndrome diagnosed in the same way as Sweet’s syndrome? Are there any differences?
Yes. MASS, including SS that has developed secondary to MDS, is diagnosed in the same way as SS. However, there can be some differences in what you might expect to find. For example:
- Differences in skin biopsy result: a biopsy taken from a SS lesion commonly shows lots of white blood cells called neutrophils. However, in MASS, other types of white blood cell, such as lymphocytes, are more likely to be seen alongside neutrophils.
- The variants histiocytoid or subcutaneous Sweet’s syndrome: sometimes, SS can occur in an unusual form and this is known as a disease variant. The variants subcutaneous and histiocytoid Sweet’s syndrome are often associated with malignancy, but not always (Nelson et al, 2017). Histiocytoid SS can sometimes be mistaken for leukaemia cutis. This is a condition where leukaemia cells infiltrate the skin causing skin lesions to develop.
- No joint pain: joint pain in MASS less likely than in SS (Marcoval et al, 2016; Nelson et al, 2017).
- Higher erythrocyte sedimentation rate (ESR): this is a blood test that detects inflammation in the body. It’s commonly raised in both SS and MASS, but tends to be higher in MASS than SS (Casarin Costa et al, 2017).
- Normal to low white blood cell count, including neutrophil count: SS normally causes a raised white blood cell and neutrophil count. In MASS, this is less likely to happen, and the white blood cell or neutrophil count may be normal or low (Casarin Costa et al, 2017; Cohen, 2007; Nelson et al, 2017).
- Anaemia (a low red blood cell count): very common in MASS, but uncommon in SS where there is no underlying cause (Cohen, 2007; Marcoval et al, 2016; Nelson et al, 2017).
- Thrombocytopenia (a low platelet count): quite common in MASS, but uncommon in SS (Cohen, 2007; Marcoval et al, 2016; Nelson et al, 2017).
Also, click here to learn more about neuro-Sweet’s disease (NSD), a neurological variant of SS. In patients with MDS, examination of cerebrospinal fluid (CSF), a clear and colourless fluid found in the brain and spinal cord, is less likely to show neutrophils or lymphocytes (Oka et al, 2017) .
Can MDS make Sweet’s syndrome more difficult to treat and manage?
Yes, MDS can make SS more difficult to manage. This is mainly because the SS often won’t settle down until the MDS is treated and brought under control, and even then, may not resolve completely.
What is the treatment for MDS-associated Sweet’s syndrome?
Corticosteroids, otherwise known as steroids, e.g. prednisone or prednisolone, are the main form of treatment for both SS and MASS. However, MASS patients, including those with MDS, don’t always respond as well to steroids as those with SS. Immunoglobulin therapy, thalidomide, or anakinra can be considered as additional or alternative treatments (Browning et al, 2005; Gill et al, 2010; Passaro et al, 2013). In patients with AML, dapsone, colchicine, and ciclosporin have been used to successfully treat MASS, even when steroids haven’t been effective (El-Khalawany et al, 2016). Ciclosporin has also been effective in low-risk MDS (Oka et al, 2017).
Read more about treatment here.
Hashemi, S., Fazeli , S., Vahedi, A. and Golabchifard, R. (2016) Rituximab for refractory subcutaneous Sweet’s syndrome in chronic lymphocytic leukemia: A case report. Molecular and Clinical Oncology, Mar;4(3):436-440 (PMC).
MDS UK Patient Support Group. Includes information on MDS symptoms, diagnosis, and treatment. Consultant Haematologist, Dr. Austin Kulasekararaj, Kings College Hospital, London, is associated with this group. He has experience of treating MASS.
NHS Choices (2018) Myelodysplastic syndrome (myelodysplasia) (online). Reviewed 22/01/18, and accessed 10/02/18. Includes information on types of MDS, symptoms, diagnosis, treatment, lenalidomide (a biological therapy), and clinical trials.
Özdoğu, H., Yeral, M., and Boğa, C. (2017) An Unusual Giant Leg Ulcer as a Rare Presentation of Sweet’s syndrome in a Patient with Hairy Cell Leukemia Successfully Managed by Splenectomy. Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology, Mar 8 (PubMed).
Rech, G., Balestri, R., La Placa, M., Magnano, M. and Girardelli, C. (2016) Single Nail Involvement as First Sign of Sweet’s Syndrome. Skin Appendage Disorders, Sep;2(1-2):61-62 (PMC). This is a case of SS developing secondary to essential thrombocythaemia.
Yaghmour, G., Wiedower, E., Yaghmour, B., Nunnery, S., Duncavage, E. and Martin, M. (2017) Sweet’s syndrome associated with clonal hematopoiesis of indeterminate potential responsive to 5-azacitidine. Therapeutic Advances in Hematology, Feb;8(2):91-95 (PMC).
Browning, C., Dixon, J., Malone, J. and Callen, J. (2005) Thalidomide in the treatment of recalcitrant Sweet’s syndrome associated with myelodysplasia. Journal of the American Academy of Dermatology, Aug; 53 (2 Suppl 1): S 135-8 (PubMed).
Chen, S., Kuo, Y., Liu, Y., Chen, B., Lu, Y. and Miser, J. (2016) Acute Myeloid Leukemia Presenting with Sweet Syndrome: A Case Report and Review of the Literature. Pediatrics and Neonatology (online).
El-Khalawany, M., Aboeldahab, S., Mosbeh, A. and Thabet, A. (2016) Clinicopathologic, immunophenotyping and cytogenetic analysis of Sweet syndrome in Egyptian patients with acute myeloid leukemia. Pathology, research and practice, Oct 26 (PubMed).
Gill, H., Leung, A., Trendell-Smith, N., Yeung, C. and Liang, R. (2010) Case Report. Sweet Syndrome due to Myelodysplastic Syndrome: Possible Therapeutic Role of Intravenous Immunoglobulin in Addition to Standard Treatment. Advances in Hematology. Article ID 328316 (Hindawi).
Guarneri, C., Wollina, U., Lotti, T., Maximov, G., Lozev, I., Gianfaldoni, S., Pidakev, I., Lotti, J. and Tchernev, G. (2018) Sweet’s Syndrome (SS) in the Course of Acute Myeloid Leukaemia (AML). Open Access Macedonian Journal of Medical Sciences, Jan 13;6(1):105-107 (PMC).
John Hopkins Medicine (2018) Myelodysplastic Syndrome. The Sidney Kimmel Comprehensive Cancer Center (online). Accessed 10/02/18. Includes US information on types of MDS, risk factors, symptoms, diagnosis, risk classification, treatment, and research.
Marcoval, J., Martin-Callizo, C., Valenti-Medina, F., Bonfill-Orti, M. and Martinez-Molina, L. (2016) Sweet syndrome: long-term follow-up of 138 patients. Clinical and Experimental Dermatology, Oct;41(7):741-6 (PubMed).
Nelson, C., Noe, M., McMahon, C., Gowda, A., Wu, B., Ashchyan, H., Perl, A., James, W., Micheletti, R. and Rosenbach, M. (2017) Sweet syndrome in patients with and without malignancy: A retrospective analysis of 83 patients from a tertiary academic referral center, Oct 26 (PubMed).
NHS Choices (2016) Acute Myeloid Leukaemia (online). Reviewed 14/03/16, and accessed 10/02/18.
Oka, S., Ono, K. And Nohgawa, M. (2017) Successful Treatment of Syndrome of Inappropriate Antidiuretic Hormone Secretion Associated with Neuro-Sweet Disease in Myelodysplastic Syndrome. Internal Medicine (Tokyo, Japan), Dec 8th (J-Stage).
Passaro, G., Cerrito, L., Giovinale, M., Marinaro, A., Soriano, A,. Rigante, D. and Manna, R. (2013) P03-019 – Anakinra for sweet syndrome treatment. Pediatric Rheumatology Online Journal, Nov; 11(Suppl 1).
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