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Case Report

Ritin Mohindra1,
Pancytopenia in Cytophagic Histiocytic
Santosh Ghai2, Panniculitis
Narender Singh Negi3,
Neelima Jain4 Abstract
1,2
Senior Resident,
3 Cytophagic histiocytic panniculitis (CHP) is a rare subtype of panniculitis that usually
Professor and Unit Head,
4
Professor, Department of
follows a fatal course, with a terminal hemophagocytic syndrome. We observed a
Medicine, Unit 3A, VMMC case of pancytopenia, generalized lymphadenopathy and panniculitis, which on
and Safdarjung Hospital, subsequent investigations (skin biopsy and bone marrow biopsy) revealed
New Delhi 110029. hemophagocytic and cytophagocytic picture.
Correspondence to:
Dr. Santosh Ghai, Keywords: Pancytopenia, Panniculitis.
Department of Medicine,
Unit 3A, VMMC and Introduction
Safdarjung Hospital, New
Delhi 110029. Cytophagic histiocytic panniculitis was first described in 1980 by Winkelmann as a
E-mail Id: chronic histiocytic disease of the subcutaneous adipose tissue.1 Cytophagic histiocytic
santosh_ghai@yahoo.com panniculitis is a rare panniculitis that is associated with systemic features including
fevers, hepatosplenomegaly, lymphadenopathy, pancytopenia, hepatic
abnormalities, hypertriglyceridemia, and coagulopathy.2,3

The panniculitis lesions show adipose tissue lymphocytic and histiocytic infiltration
along with hemophagocytosis, which may also appear in bone marrow, spleen,
lymph nodes, and liver. Patients may have a rapidly fatal disease course, a longer
disease course with intermittent remissions and exacerbations for many years prior
to death, or a nonfatal acute or intermittent course responsive to treatment. The
cytophagocytic disorder in these patients is a hemophagocytic lymphohistiocytosis
(HLH). This syndrome has also been described in patients with T-cell lymphoma.4

Case Report
A 15-year-old male, resident of Uttar Pradesh, presented with chief complaints of
fever, loss of appetite, malaise and weight loss for the last 1 month. There was a
complaint of recurrent skin rashes which were reddish, painful that progressed to
ulceration on bilateral lower limb. No other systemic features were present.

On examination, the patient was febrile. His vitals were temperature 101°F, pulse
92/min and blood pressure 110/70 mmHg. Severe pallor was present. Bilateral
multiple discrete cervical, axillary and inguinal lymph nodes were palpable. Per
abdomen examination revealed palpable liver 2 cm below right costal margin non-
tender and spleen palpable 2 cm below left costal margin. Local examination of
bilatreral lower limb revealed erythematous nodule on right lower limb and another
How to cite this article: ulcerated lesion on right thigh of the patient suggestive of subcutaneous nodules.
Mohindra R, Ghai S, Negi NS
et al. Pancytopenia in Preliminary investigation of patient revealed pancytopenia with mild derangement in
Cytophagic Histiocytic LFT and coagulation profile.
Panniculitis. J Adv Res Med
2016; 3(2&3): 11-13. Hb-3.6 g/dL, TLC 3000/mm3, DLC 27.5%, neutrophils 47.2%, lymphocytes 8.1%,
monocytes 11.3%, eosinophils 5.9%, basophils MCV 78 fl, platelet count was
ISSN: 2349-7181 <10000/mm3, LFT showed total bilirubin 1.3 (0.5/0.8), SGOT 28, SGPT 27, ALP 59,
Serum Na+ 135, K+ 4.1, PT 23.0 sec, APTT 28.9 sec, and INR 1.9 B. Urea 42 mg%, S.Cr.

© ADR Journals 2016. All Rights Reserved.


Mohindra R et al. J. Adv. Res. Med. 2016; 3(2&3)

7 mg%, Repeat CBC showed Hb 3.7g/dL, TLC count differed in that systemic signs and symptoms were
1500/mm3, neutrophils 40%, lymphocytes 40%, lacking, and cytophagia was not evident in
monocytes 13.3%, eosinophils 6.7%, reticulocyte count extracutaneous organs.
0.19%, and peripheral smear showed predominantly
normocytic normochromic picture. Serological markers With the widespread use of immunocytochemistry to
including HIV were non-reactive. Urine routine characterize cellular infiltrates, the existence of
microscopy and chest X-ray were normal. Ultrasound malignant T-cell proliferations mimicking inflammatory
abdomen showed mild hepatosplenomegaly. panniculitis has been recognized.4,8,11

Biopsy taken from skin lesion showed lobular Gonzales et al. first described T-cell lymphoma involving
panniculitis with predominance of macrophages. subcutaneous tissue as a rare, distinct subset of
Infiltrates of lymphocytes and histiocytes, many of peripheral T-cell lymphoma (PTL) characterized by a
which contained phagocytosed leucocytes and propensity to be associated with a hemophagocytic
erythrocytes; no atypical cells were noted. syndrome and by an aggressive clinical course.

Bone marrow aspiration and biopsy was done which CHP is generally treated with systemic corticosteroids.
revealed marked predominance of lymphocytes and Methyl-prednisolone pulse therapy has been advocated
histiocytosis with active erythrophagocytosis. as a treatment option. Alternate immunosuppressive
therapy, such as cyclosporine, maybe used as a first-line
Thus based on clinical and pathological examination, a treatment.12 Ito et al. reported a case with CHP treated
diagnosis of haemophagocytic syndrome was made. successfully with a combination of cytotoxic drugs
(CHOP-cyclophosphamide, doxorubicin, vincristine and
Discussion prednisolone) and cyclosporine.13 In severe relapse
cases, high-dose chemotherapy followed by autologous
The diagnosis of haemophagocytic syndrome is based peripheral blood stem cell transplantation can be
on clinical and pathological features. Histopathology considered an alternative treatment option.14 CHP, thus,
shows inflammation of subcutaneous tissue, involving is a rare and often fatal form of panniculitis with
macrophages and T lymphocytes. Erythrophagocytosis multisystem involvement. However, it can also present
and cytophagocytosis have been described in in a benign form involving only the subcutaneous tissue,
subcutaneous tissue and internal organs, particularly thus having a broad clinical spectrum.
the lymph nodes, spleen, liver, and bone marrow.4,5
Conflict of Interest: None
Hemophagocytic syndromes represent a severe
hyperinflammatory condition with the cardinal References
symptoms of prolonged fever, cytopenias,
hepatosplenomegaly, and hemophagocytosis by 1. Winkelmann RK, Bowie EJ. Hemorrhagic diathesis
activated, morphologically benign macrophages. associated with benign histiocytic, cytophagic
Impaired function of natural killer (NK) cells and panniculitis and systemic histiocytosis. Arch Intern
cytotoxic T-cells (CTL) is characteristic for both genetic Med Nov 1980; 140(11):1460-63.
and acquired forms of hemophagocytic syndromes. 2. Aronson IK, Worobec SM. Cytophagic histiocytic
Frequent triggers are infectious agents, mostly viruses panniculitis and hemophagocytic lymphohistiocy
of the herpes group. Malignant lymphomas, especially tosis: An overview. Dermatol Ther Aug 2010; 23(4):
in adults, may be associated with hemophagocytic 389-402.
syndromes.6,7 Hemophagocytic syndromes mostly 3. Crotty CP, Winkelmann RK. Cytophagic histiocytic
follow a fatal outcome, but few case reports have panniculitis with fever, cytopenia, liver failure, and
shown non-fatal disease course. Various articles have terminal hemorrhagic diathesis. J Am Acad
also described their association with T-cell lymphomas. Dermatol Feb 1981; 4(2): 181-94.
4. Marzano AV, Berti E, Paulli M et al. Cytophagic
Crotty and Winkelmann described five patients with histiocytic panniculitis and subcutaneous
multiple inflammatory subcutaneous nodules who panniculitis-like T-cell lymphoma: report of 7 cases.
experienced a terminal hemorrhagic diathesis after a Arch Dermatol Jul 2000; 136(7): 889-96.
clinical course of 6 months to 10 years.3 5. Manoj J, Kaliyadan F, Unni M et al. Cytophagic
Histiocytic Panniculitis: Report of Two Cases. Indian
Single reports of patients with CHP who have not
J Dermatol 2011; 56(3): 306-308.
experienced a fatal outcome 5, 16, and 28 years after
the onset of the disease.8-10 This nonfatal form of CHP

ISSN: 2349-7181 12
J. Adv. Res. Med. 2016; 3(2&3) Mohindra R et al.

6. Weitzman S. Approach to hemophagocytic Dermatol Feb 1997; 36(2): 285-89.


syndromes. ASH Educ Program Book Dec 2011; 12. Ostrov BE, Athreya BH, Eichenfield AH et al.
2011(1): 178-83. Successful treatment of severe cytophagic
7. Janka GE. Hemophagocytic syndromes. Blood Rev histiocytic panniculitis with cyclosporine A. Semin
Sep 2007; 21(5): 245-53. Arthritis Rheum Jun 1996; 25(6): 404-13.
8. Gonzales CL, Medeiros J, Braziel RM et al. T-cell 13. Ito M, Ohira H, Miyata M et al. Cytophagic
lymphoma involving subcutaneous tissue: a histiocytic panniculitis improved by combined CHOP
clinicopathologic entity commonly associated with and cyclosporin A treatment. Intern Med Tokyo Jpn
hemophagocytic syndrome. Am J Surg Pathol 1991: Mar 1999; 38(3): 296-301.
1517-27. 14. Koizumi K, Sawada K, Nishio M et al. Effective high-
9. Barron DR, Davis BR, Pomeranz JR et al. Cytophagic dose chemotherapy followed by autologous
histiocytic panniculitis: a variant of malignant peripheral blood stem cell transplantation in a
histiocytosis. Cancer 1985; 55: 2538-42. patient with the aggressive form of cytophagic
10. White Jr JW, Winkelmann RK. Cytophagic histiocytic histiocytic panniculitis. Bone Marrow Transplant Jul
panniculitis is not always fatal. J Cutan Pathol 1989; 1997; 20(2): 171-73.
16: 137-44. Date of Submission: 01st Feb. 2016
11. Von den Driesch P, Staib G, Simon J et al.
Subcutaneous T-cell lymphoma. J Am Acad Date of Acceptance: 03rd Feb. 2016

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