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Cutaneous Exophytic Infiltration in Myelofibrosis

Submitted by Agnese Comelli and Ilaria Izzo

Published October 6, 2019

A 69 year old woman with idiopathic myelofibrosis presented with a 6 month history of a circular, bluish, exophytic infiltration on her anterior left tibia (6 cm in diameter) (Panel A).  The patient complained of severe pain, only partially controlled with NSAIDs. She was followed at the vascular surgery outpatient clinic and received periodic debridement and dressing of the wound.  Left leg doppler ultrasound ruled out venous insufficiency and arterial disease. The lesions were characterized by sporadic but severe bleeding with spontaneous, subsequent reepithelialisation.     

Despite close follow up, she presented to the Emergency Department at our institution with fever and inflamed, edematous skin surrounding the ulcerated lesion (Panel B).  Piperacillin/tazobactam was administered. Antibiotic therapy was switched after 12 days to tigecycline and clindamycin due to lack improvement on the initial antibiotic regimen.  The change resulted in defervescence and gradual healing of the skin ulcer. Despite resolution of the underlying infection, the circular, bluish, exophytic infiltration persisted.  Biopsy of the lesion was performed and immunohistochemical exam of tissue revealed several foci of extramedullary erythropoiesis with MPO+ CD34- myeloid precursors, erythroblasts (glycophorin C+) and rare megakaryocytes (LAT+).  The patient was subsequently diagnosed with cutaneous extramedullary erythropoiesis, a rare manifestation of myelofibrosis(1).


Panel A


Panel B

Question:

Which is the first line treatment of extramedullary erythropoiesis?

  1. Chemotherapy

  2. Cryotherapy

  3. Radiation therapy

  4. Surgical excision

Answer and Discussion

Answer C (Radiation Therapy)

Primary myelofibrosis is a myeloproliferative neoplasm that usually occurs in middle-aged and older adults. Patients usually present with fatigue and an enlarged spleen; a smaller number have complaints of weight loss, low-grade fever, bone pain, and night sweats.  The diagnostic approach includes obtaining a peripheral smear (with the presence of granulocyte precursors, nucleated and teardrop-shaped red cells being suggestive of the disease) as well as bone marrow aspiration and biopsy with molecular testing. Chemotherapy, radiation and symptom-based interventions such splenectomy and blood transfusions are used to cure or control myelofibrosis progression (2). 

Foci of extramedullary hematopoiesis may occur in almost any organ while involvement of the skin is rare.  Radiotherapy is the first line therapy for cutaneous extramedullary erythropoiesis, as hematopoietic tissue is known to be sensitive to low doses of radiation.  The role of such treatment in treating patients has been well established in non-hepatosplenic extramedullary hematopoiesis (1).

References:

 [1] Koch CA, Li CY, Mesa RA, et al. Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc 2003;78:1223-33. 

[2] Tefferi, A Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management. Am. J Hematol 2016;91: 1262–1271. 

 

Author Bios:

Agnese Comelli is a resident in Infectious and Tropical Disease at University Department of Spedali Civili of Brescia, Italy. She attended medical school at University of Medical Studies in Pavia, Italy. 

Ilaria Izzo is a consultant at University Department of Spedali Civili of Brescia, Italy, where she completed her residency. 



 

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