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An elderly man with a violaceous nodule and anemia
2 Department of Pathology, BLDEA's SBMP Medical College, Hospital & Research Centre, Bijapur, Karnataka, India
Arun C Inamadar
Department of Dermatology, Venereology and Leprosy, BLDEA's SBMP Medical College, Hospital & Research Centre, Bijapur-586103, Karnataka
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Palit A, Inamadar AC, Athanikar S B, Sampagavi V V, Deshmukh N S, Yelikar B R. An elderly man with a violaceous nodule and anemia. Indian J Dermatol Venereol Leprol 2005;71:376-378
A 60-year-old agricultural worker, admitted to the surgical ward for bleeding piles of long duration, was referred for evaluation of his skin lesions. He had developed a painful nodule on his left thigh for the past 4 weeks, which had been enlarging rapidly since its onset.
On general physical examination, the patient was pale and emaciated. There were multiple ecchymotic patches over both forearms and legs. A violaceous, shiny nodular lesion of about 4 cm x 4 cm was observed on the extensor aspect of his left thigh [Figure - 1]. The lesion was firm, non-compressible, movable over the underlying structures and slightly tender. Systemic examination revealed hepatosplenomegaly.
A complete hemogram revealed Hb, 7 gm%; TLC, 11,300/cmm; differential count: neutrophils, 42%; lymphocytes, 52%; eosinophils, 4%; and monocytes, 2%; platelet count 60,000/cmm; and ESR, 120 mm at the end of the 1st hour. The peripheral blood smear showed predominantly normocytic, normochromic, few hypochromic, microcytic and nucleated RBCs. A few large cells with atypical morphology were observed.
The Giemsa-stained photomicrograph of the aspirated material from the nodule has been presented in [Figure - 2].
What is the diagnosis?
Diagnosis: 0 Granulocytic sarcoma associated with acute myeloid leukemia The Giemsa-stained FNAC smear showed clusters of blast cells [Figure - 2]. The PAP-stained smear showed large myeloid precursor cells with coarse granules, suggestive of myeloblasts [Figure - 3]. A differential count revealed myeloblasts, 60%; neutrophils, 26%; lymphocytes, 10%; and monocytes, 4%.
The patient was referred to the Medicine Department for further work-up and bone marrow aspiration studies. He refused further invasive investigations and left the hospital against medical advice.
Granulocytic sarcoma (GS) is a rare tumor resulting from extramedullary invasion of granulocyte precursor cells in patients with acute myeloid leukemia (AML). It is variably known as myeloid sarcoma, myeloblastoma or chloroma. The tumors are usually localized to the bone, periosteum, soft tissue, lymph node or skin. The commonest extracutaneous sites are skull bones, orbit and paranasal sinuses, but involvement of intracranial structures, viscera, serous membranes, breast and salivary glands has been reported. When the skin is involved, it constitutes a specific form of leukemia cutis. The lesion occurs as a solitary, rapidly enlarging nodule, occasionally with a greenish hue.
The overall incidence of GS varies from 2% to 14%. It is common in children and younger patients with AML. GS may occur in three clinical situations:
· In patients with AML,
· Rarely, in patients with myelodysplastic syndrome with leukemic transformation or chronic myeloid leukemia with impending blast crisis, or
· In patients without hematological or bone marrow evidence of acute myeloid leukemia (aleukemic leukemia cutis).
The occurrence of GS in patients with AML is a poor prognostic factor, indicating a life expectancy of one year in 90% of the cases. In patients of myelodysplastic syndrome with GS, there is a definite progression to AML. The majority of patients with aleukemic leukemia and GS develop overt AML within a mean period of 10 months.
The pathogenesis of GS is unknown. It has been speculated that trauma induced extravasation of myeloid precursor cells in the skin leads to their localized replication. Recently, the role of the neural cell adhesion molecule CD56 has been highlighted. Co-expression of CD56 and CD4 on the tumor cells and co-existent 8:21 chromosome translocation have been implicated as possible risk factors for the development of GS in patients with AML.
The earlier name chloroma was derived from the greenish color of some of the tumors which results from the increased level of myeloperoxidase enzymes in the immature cells. The color can be enhanced by rubbing the tumor with alcohol swabs. It also fluoresces red under ultraviolet light due to the presence of protoporphyrin. The diagnosis can be confirmed by biopsy, dried imprint smears and cytological preparations.
Histopathological features include a dense population of myeloblasts, myelocytes and some mature cells infiltrating the collagen fibers and deeper tissues. Focal necrosis may be present. The nature of the granules (eosinophilic/neutrophilic) may indicate the cells′ myeloid lineage. Peripheral blood and bone marrow examinations are essential for management. Neutropenia is common in patients with GS.
The differential diagnosis includes cutaneous B-cell lymphoma, which may clinically present as a single or multiple violaceous nodules involving the head, neck or trunk. Histopathologically, there is a bottom-heavy infiltrate of lymphocytes sparing the epidermis. In some cases, immunohistochemistry, flow-cytometry or cytochemical stains may be required to exclude lymphoma. Pseudochloroma is a rare condition characterised by the collection of normal hemopoietic tissue in unusual locations. It can be differentiated from GS by the constituent mature cells.
No established treatment is available for individual lesions of GS. The lesions are radiosensitive. Radiotherapy and electron-beam therapy are palliative and causes rapid shrinkage of the lesions. In view of the frequent subsequent relapse, systemic chemotherapy, as recommended for the underlying hematological condition, is the preferred mode of treatment.
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