Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obervation Letter
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Quiz
2005:71:5;376-378
doi: 10.4103/0378-6323.16802
PMID: 16394476

An elderly man with a violaceous nodule and anemia

Aparna Palit1 , Arun C Inamadar1 , SB Athanikar1 , VV Sampagavi1 , NS Deshmukh1 , BR Yelikar2
1 Department of Dermatology, Venereology, Leprosy, BLDEA's SBMP Medical College, Hospital & Research Centre, Bijapur, Karnataka, India
2 Department of Pathology, BLDEA's SBMP Medical College, Hospital & Research Centre, Bijapur, Karnataka, India

Correspondence Address:
Arun C Inamadar
Department of Dermatology, Venereology and Leprosy, BLDEA's SBMP Medical College, Hospital & Research Centre, Bijapur-586103, Karnataka
India
How to cite this article:
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
Copyright: (C)2005 Indian Journal of Dermatology, Venereology, and Leprology

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.

Discussion

Granulocytic sarcoma (GS) is a rare tumor resulting from extramedullary invasion of granulocyte precursor cells in patients with acute myeloid leukemia (AML).[1] 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.[1] 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.[1] 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%.[1] It is common in children and younger patients with AML. GS may occur in three clinical situations:[2]

· 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.[3] 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.[3]

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.[3] 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.[2]

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.[4] The color can be enhanced by rubbing the tumor with alcohol swabs.[3] It also fluoresces red under ultraviolet light due to the presence of protoporphyrin.[5] 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.[1] 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.[1]

The differential diagnosis includes cutaneous B-cell lymphoma,[6] 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.[6] Pseudochloroma is a rare condition characterised by the collection of normal hemopoietic tissue in unusual locations.[5] 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.[1] Radiotherapy and electron-beam therapy are palliative and causes rapid shrinkage of the lesions.[3] In view of the frequent subsequent relapse, systemic chemotherapy, as recommended for the underlying hematological condition, is the preferred mode of treatment.

References
1.
Greer JP, Baer MR, Kinney MC. Acute myeloid leukemia in adults. In: Greer JP, Foerster J, Lukens JN, Rodgers GM, Paraskevas F, Glader B, editors. Wintrobe's Clinical hematology. 11th edn. Philadelphia: Lippincott, Williams and Wilkins; 2004. p. 2097-142.
[Google Scholar]
2.
Murakami Y, Nagae S, Matsuishi E, Irie K, Furue M. A case of CD 56+ cutaneous aleukemic granulocytic sarcoma with myelodysplastic syndrome. Br J Dermatol 2000;143:587-90.
[Google Scholar]
3.
Harris DW, Ostlere LS, Rustin MH. Cutaneous granulocytic sarcoma (chloroma) presenting as the first sign of relapse following autologous bone marrow transplantation for acute myeloid leukemia. Br J Dermatol 1992;127:182-4.
[Google Scholar]
4.
Schwartz RA. Cutaneous metastatic disease. J Am Acad Dermatol 1995;33:161-82.
[Google Scholar]
5.
Ashley DJ. Myeloid leukemia and allied disorders. In: Ashley DJB, editor. Evans' Histopathological appearances of tumors, 4th edn. Edinburgh: Churchill Livingstone; 1990. p. 191-204.
[Google Scholar]
6.
Zic JA, Kiripolsky MG, Hamilton KS, Greer JP. Cutaneous T-cell lymphomas, mycosis fungoides and Sιzary syndrome. In: Greer JP, Foerster J, Lukens JN, Rodgers GM, Paraskevas F, Glader B, editors. Wintrobe's Clinical hematology, 11th edn. Philadelphia: Lippincott, Williams and Wilkins; 2004. p. 2485-520.
[Google Scholar]

Fulltext Views
146

PDF downloads
45
Show Sections