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Case Report

Ritin Mohindra1,
Pancytopenia in Cytophagic Histiocytic
Santosh Ghai2, Panniculitis
Narender Singh Negi3,
Neelima Jain4 Abstract
Senior Resident,
3 Cytophagic histiocytic panniculitis (CHP) is a rare subtype of panniculitis that usually
Professor and Unit Head,
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 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
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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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