Case Report
Ritin Mohindra1,
Santosh Ghai2,
Narender Singh Negi3,
Neelima Jain4
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
Medicine, Unit 3A, VMMC
and Safdarjung Hospital,
New Delhi 110029.
Correspondence to:
Dr. Santosh Ghai,
Department of Medicine,
Unit 3A, VMMC and
Safdarjung Hospital, New
Delhi 110029.
E-mail Id:
santosh_ghai@yahoo.com
How to cite this article:
Mohindra R, Ghai S, Negi NS
et al. Pancytopenia in
Cytophagic Histiocytic
Panniculitis. J Adv Res Med
2016; 3(2&3): 11-13.
ISSN: 2349-7181
© ADR Journals 2016. All Rights Reserved.
Pancytopenia in Cytophagic Histiocytic
Panniculitis
Abstract
follows a fatal course, with a terminal hemophagocytic syndrome. We observed a
case of pancytopenia, generalized lymphadenopathy and panniculitis, which on
subsequent investigations (skin biopsy and bone marrow biopsy) revealed
hemophagocytic and cytophagocytic picture.
Keywords: Pancytopenia, Panniculitis.
Introduction
Cytophagic histiocytic panniculitis was first described in 1980 by Winkelmann as a
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
ulcerated lesion on right thigh of the patient suggestive of subcutaneous nodules.
Preliminary investigation of patient revealed pancytopenia with mild derangement in
LFT and coagulation profile.
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
<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.
Mohindra R et al.
7 mg%, Repeat CBC showed Hb 3.7g/dL, TLC count
1500/mm3, neutrophils 40%, lymphocytes 40%,
monocytes 13.3%, eosinophils 6.7%, reticulocyte count
0.19%, and peripheral smear showed predominantly
normocytic normochromic picture. Serological markers
including HIV were non-reactive. Urine routine
microscopy and chest X-ray were normal. Ultrasound
abdomen showed mild hepatosplenomegaly.
Biopsy taken from skin lesion showed lobular
panniculitis with predominance of macrophages.
Infiltrates of lymphocytes and histiocytes, many of
which contained phagocytosed leucocytes and
erythrocytes; no atypical cells were noted.
Bone marrow aspiration and biopsy was done which
revealed marked predominance of lymphocytes and
histiocytosis with active erythrophagocytosis.
Thus based on clinical and pathological examination, a
diagnosis of haemophagocytic syndrome was made.
Discussion
The diagnosis of haemophagocytic syndrome is based
on clinical and pathological features. Histopathology
shows inflammation of subcutaneous tissue, involving
macrophages and T lymphocytes. Erythrophagocytosis
and cytophagocytosis have been described in
subcutaneous tissue and internal organs, particularly
the lymph nodes, spleen, liver, and bone marrow.4,5
Hemophagocytic syndromes represent a severe
hyperinflammatory condition with the cardinal
symptoms of prolonged fever, cytopenias,
hepatosplenomegaly, and hemophagocytosis by
activated, morphologically benign macrophages.
Impaired function of natural killer (NK) cells and
cytotoxic T-cells (CTL) is characteristic for both genetic
and acquired forms of hemophagocytic syndromes.
Frequent triggers are infectious agents, mostly viruses
of the herpes group. Malignant lymphomas, especially
in adults, may be associated with hemophagocytic
syndromes.6,7 Hemophagocytic syndromes mostly
follow a fatal outcome, but few case reports have
shown non-fatal disease course. Various articles have
also described their association with T-cell lymphomas.
Crotty and Winkelmann described five patients with
multiple inflammatory subcutaneous nodules who
experienced a terminal hemorrhagic diathesis after a
clinical course of 6 months to 10 years.3
Single reports of patients with CHP who have not
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
J. Adv. Res. Med. 2016; 3(2&3)
differed in that systemic signs and symptoms were
lacking, and cytophagia was not evident in
extracutaneous organs.
With the widespread use of immunocytochemistry to
characterize cellular infiltrates, the existence of
malignant T-cell proliferations mimicking inflammatory
panniculitis has been recognized.4,8,11
Gonzales et al. first described T-cell lymphoma involving
subcutaneous tissue as a rare, distinct subset of
peripheral T-cell lymphoma (PTL) characterized by a
propensity to be associated with a hemophagocytic
syndrome and by an aggressive clinical course.
CHP is generally treated with systemic corticosteroids.
Methyl-prednisolone pulse therapy has been advocated
as a treatment option. Alternate immunosuppressive
therapy, such as cyclosporine, maybe used as a first-line
treatment.12 Ito et al. reported a case with CHP treated
successfully with a combination of cytotoxic drugs
(CHOP-cyclophosphamide, doxorubicin, vincristine and
prednisolone) and cyclosporine.13 In severe relapse
cases, high-dose chemotherapy followed by autologous
peripheral blood stem cell transplantation can be
considered an alternative treatment option.14 CHP, thus,
is a rare and often fatal form of panniculitis with
multisystem involvement. However, it can also present
in a benign form involving only the subcutaneous tissue,
thus having a broad clinical spectrum.
Conflict of Interest: None
References
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associated with benign histiocytic, cytophagic
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Date of Submission: 01st Feb. 2016
Date of Acceptance: 03rd Feb. 2016
ISSN: 2349-7181