Cutaneous extramedullary hematopoiesis in idiopathic myelofibrosis

Abstract

A 67‐year‐old German woman presented with a 2‐month history of indolent erythematous macules and livid papulonodules of 0.5–1.5 cm in diameter scattered on her abdomen (Fig. 1). On physical examination, the patient was alert, the liver was palpable 3 cm below the right costal margin, and the spleen was enlarged 3 cm below the left costal margin. There was no lymphadenopathy. 1 Clinical lesions with extramedullary hematopoiesis on the abdomen Laboratory findings disclosed the following abnormalities: hemoglobin, 10.6 g/dL; hematocrit, 32.4%; white blood cell count, 4.600/mm3 myelocytes, with 15.2% lymphocytes, 74.1% neutrophils, 6.7% monocytes, and 3.6% eosinophils. Repeated bone marrow biopsy specimens showed severe myelofibrosis and osteosclerosis. Histologic examination of an incisional skin biopsy specimen revealed a mixed dermal infiltrate containing numerous histiocytic and myelocytic cells that resembled atypical megakaryocytes. Cells of the erythroid series were absent. The giant cells were labeled with CD61, thereby supporting the megakaryocytic lineage. The naphthol As‐D chloracetate reaction was positive on myelocytic cells of the infiltrate indicating granulocytopoiesis (Fig. 2). 2 Mixed dermal infiltrate composed of histiocytic, myelocytic, and multinuclear cells. Multinuclear cells were labeled with CD61 (upper right inset) and were negative for naphthol As‐D chloracetate reaction, which detected myelocytic cells (lower left inset) The patient had received electron‐beam irradiation 8 months previously, and had been treated with hydroxyurea (Litalir®, 500 mg/day) for 8 months before the manifestation of myelofibrosis on the skin. Two months after her first visit to our out‐patient clinic, the eruptions expanded, and electron‐beam irradiation was considered, but not performed, because of the progress of the disease. The patient's course was complicated by severe bone pain, the development of anemia (2.6 g/dL), a blast crisis with 80% undifferentiated, myelocytic blasts, and thrombocytopenia (26 × 109/L). Symptomatic treatment, such as several blood transfusions and analgesic agents, did not stabilize the situation. During this stage of the illness, the skin alterations remained unchanged. Sixteen months after the first diagnosis of myelofibrosis, the patient died due to conversion into chronic myelogenous leukemia, as shown by repetition of the bone marrow aspiration and the peripheral blood count.

Cutaneous extramedullary hematopoiesis in a patient with idiopathic myelofibrosis

“…By 2005, there had been 29 cases (eight females and 21 males) of CEH reported worldwide (Table 1). Eighteen cases had been reported by 1990 2–18 and 10 new cases were reported in the proceeding 15 years 19–25 . The average age of the patient was 60 years old.…”

“…Eighteen cases had been reported by 1990 2-18 and 10 new cases were reported in the proceeding 15 years. [19][20][21][22][23][24][25] The average age of the patient was 60 years old. The youngest patient was a 45-year-old Japanese man 11 and the oldest was an 84-year-old man.…”

“…The youngest patient was a 45‐year‐old Japanese man 11 and the oldest was an 84‐year‐old man 22 . Most of these cases were of primary myelofibrosis, 2,3,5,6–12,15–21,23–25 except two for cases of polycythemia vera, 4,14 four cases of agnogenic myeloid metaplasia 22 and one case of acute myelofibrosis 17 . The two cases of polycythemia vera were finally diagnosed as myelofibrosis 4,14 .…”

A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

“…There seems to be no association between timing of skin manifestations and time to disease progression in the reported cases. Yet, there are reports of patients with blastic transformation shortly after development of skin manifestations, which may indicate that skin involvement may serve as a sign of disease acceleration or development of blastic crisis [13,14]. In almost half of the cases, splenectomy preceded the occurrence of skin lesions but whether splenectomy has an effect on the development or progression of EMH is not clear.…”

Cutaneous Hematopathology

“…Literature documentation of CEMH comprises a total of about 30 cases and appears to be most frequently associated with myelofi brosis or myeloproliferative disorders (Patel et al 1995 ;Revenga et al 2000 ;Fernandez Acenero et al 2003 ). Increased levels of transforming growth factor-beta appear to be associated with CEMH in patients with idiopathic myelofi brosis ( Collie et al 2013 ;Corella et al 2008 ;Haniffa et al 2006 ;Kawakami et al 2008 ;Kwon et al 1999 ;Lane et al 2002 ;Miyata et al 2008 ;Mizoguchi et al 1990 ;Pagerols et al 1998 ;Rodriguez et al 1991 ;Rogalski et al 2002 ;Ruberto et al 1995 ).…”