Nalini Calton

Acute interstitial nephritis in association with polymyositis

170 J Postgrad Med April 2008 Vol 54 Issue 2 lower lobes of the lungs, especially if it is in contact with the diaphragm, along with primary lung, pleural and diaphragmatic neoplasms as well as metastases and other non-neoplastic causes. A noninvasive imaging modality such as a CT scan with FNAC should establish the correct diagnosis and more invasive procedures like diagnostic pneumoperitoneum and an exploratory thoracotomy should seldom be required.

Metastatic squamous cell carcinoma of the lung masquerading as a soft tissue tumor

Carcinoma of lung can metastasize to any organ system; however, metastasis to skeletal muscles is extremely rare. A 63-year-old man, known case of pulmonary tuberculosis on treatment, presented with a painful swelling in his left leg. Examination revealed a 5.0 cm × 3.0 cm calf swelling, which on imaging was suggestive of a soft tissue tumor. Fine-needle aspiration cytology of the swelling revealed it to be squamous cell carcinoma. Further investigations revealed a mass in the left lower lobe of the lung. Biopsies from both the lung lesion and calf swelling confirmed the diagnosis of squamous cell carcinoma of lung with metastasis to the calf muscle. The case is being presented because of its unusual presentation and rarity.

T Cell Prolymphocytic Leukemia (T-PLL): Cutaneous Involvement in an Uncommon Leukemia

A 70 years old lady presented with multiple neck swellings and abdominal pain for 6 months. Examination showed pallor with bilateral cervical, axillary and inguinal lymphadenopathy. There was moderate hepatomegaly with no splenomegaly. She also had a macular rash over the trunk and limbs. Chest X-ray was normal. Ultrasonogram of the abdomen showed prominent peri-portal lymphadenopathy. Investigations showed hemoglobin of 84 g/l, total leukocyte count of 311 9 10/l and platelets of 119 9 10/ l. Differential count showed 94% lymphoid cells, 4% neutrophils and 2% metamyelocytes. The lymphoid cells were of size 10–14 lm with indented and clefted nuclear outlines, single prominent nucleolus and scanty to moderate cytoplasm (Fig. 1a).Many of the cells showed cerebriform morphology with cytoplasmic blebs in few. Bone marrow aspirate was hypercellular with trilineage hematopoietic depression and diffuse replacement by lymphoid cells with clumped chromatin, inconspicuous nucleoli and scant basophilic cytoplasm. Bone marrow differential count showed: 79% lymphoid cells with few residual erythroid and myeloid hematopoietic cells. Trephine biopsy showed a diffuse infiltrate of lymphoid cells (Fig. 1b). Cytochemistry was not done due to non availability of stains. Immunophenotyping showed lymphoid cells to be positive for CD45, CD3 (93.2%), CD5, CD4 and CD7. CD8, B-cell and myeloid markers were negative. CD52 expression was not studied. Biochemical investigations were normal. Biopsy from the macular rash showed dense peri-appendageal lymphocytic dermal infiltrate with melanin incontinence and no epidermotropism (Fig. 1c). The infiltrate was positive for CD3 (Fig. 1d). CD20 was negative with moderately high (40%) Ki67 index. A diagnosis of T-cell Prolymphocytic leukaemia (T-PLL) was made. The patient was started on Fludarabine and Cyclophosphamide. After the third cycle of chemotherapy, she developed tumor lysis syndrome with renal failure and an infarct in the left lentiform nucleus. In view of the poor prognosis, the relatives took her against medical advice. T-PLL is an uncommon chronic lymphoproliferative disorder with male preponderance and comprises 2% of mature lymphocytic leukemias. The median age at presentation is 65 years. Lymphadenopathy and hepatosplenomegaly are common with skin lesions and effusions seen in 20 and 15% of patients respectively [1, 2]. Patients have leukocytosis with one thirds having associated anemia and thrombocytopenia. Liver function tests are frequently abnormal with raised urate and lactate dehydrogenase levels [2]. Peripheral blood smear shows prolymphocytes with classical T-PLL seen in two-thirds of patients with larger lymphoid cells with irregular outline and cytoplasmic blebs. Remaining patients show small lymphoid cells or have cerebriform nuclei [2–4]. Skin lesions are often seen without epidermotropism [5]. Immunophenotyping in T-PLL shows the most frequent phentotype to be CD4?, CD8(65% cases), CD4?/CD8? (25% cases) and CD8?/CD4(10% cases). Positivity for CD2, CD3, CD5 and CD7 is consistently seen. CD52 expression is usually seen and can be used for targeted therapy with Alemtuzumab. Tdt is negative along with pan B cell markers. Cytogenetic abnormalities seen commonly in T-PLL are t(14;14)(q11;q32) and inv(14). The & Naveen Kakkar kakkar.naveen@gmail.com

Systemic and primary cutaneous anaplastic large cell lymphoma: Clinical features, morphological spectrum, and immunohistochemical profile

Background: T-cell lymphomas with anaplastic morphology typically comprise of anaplastic lymphoma kinase positive, anaplastic large cell lymphoma (ALK+ ALCL), ALK-negative ALCL (ALK- ALCL), and primary cutaneous ALCL (PC-ALCL). However, other entities such as diffuse large B-cell lymphoma, peripheral T-cell lymphoma, Hodgkin lymphoma, and undifferentiated carcinoma can also show similar anaplastic features. Aims: To study the clinical features and histological spectrum of ALCL and emphasize the role of immunohistochemistry (IHC) in their diagnosis and categorization. Setting and Design: Eight cases of ALCL diagnosed over a period of 4 years were selected for the study. Materials and Methods: Histopathological review and IHC was performed on all cases. Two ALK+ ALCL cases were tested by fluorescent in situ hybridization (FISH) for t(2;5)(p23;q35). Results: There were four cases of ALK+ ALCL and two each of ALK- ALCL and PC-ALCL. Histologically, all the subtypes showed pleomorphic and “hallmark” cells with strong CD30 expression and variable loss of T-cell antigens. One case of PC-ALCL was leukocyte common antigen (LCA) negative. Epithelial membrane antigen was positive in all the six systemic ALCL cases. Two cases tested for t(2;5)(p23;q35) by FISH were positive. Conclusions: Diagnosis of ALCL is based on recognizing the key morphological features, especially the presence of “hallmark” cells. IHC is essential for confirmation of diagnosis and excluding other malignancies with anaplastic morphology. The inclusion of CD30 in the initial IHC panel will help identify LCA negative cases and avoid misdiagnosis.

Mullerian choristoma in a case of spinal dysraphism.

It is extremely rare to find mullerian choristomas in association with spinal dysraphism, with <10 cases published in English literature. We report a case of heterotopic uterus and fallopian tube-like tissue within a lumbar subcutaneous lipoma associated with spina bifida and tethered cord. A 21-year-old lady presented with lumbar swelling since birth and dull pain in the lower back. Magnetic resonance imaging showed spina bifida at level L3 and L4, tethering of the cord and a subcutaneous lipomatous swelling. Biopsy revealed lobules of fibroadipose tissue embedded in which were seen organoid cystic structures containing prominent smooth muscle coats in their wall. These cystic structures were lined by the endometrium and showed fallopian tube-like papillary infoldings. Immunohistochemistry showed estrogen receptor positivity in the epithelium, stroma, and smooth muscles. The epithelial cells were also positive for cancer antigen 125 and cytokeratin 7 while the stromal cells showed CD10 positivity, supporting mullerian derivation. The pathogenesis and differential diagnosis of such lesions is discussed.

New onset bullous lupus erythematosus in a systemic lupus erythematosus patient after initiation of hydroxychloroquin

We report a case of a 14-year-old Indian girl, a diagnosed case of systemic lupus erythematosus (SLE) who developed generalized erythematous rash and joints pain for 15 days. She was prescribed hydroxychloroquin and low dose deflazacort for joint pains. Within 3 days of initiation of hydroxychloroquin she developed generalized eruptions in the form of tense, fluid-filled blisters, erosions, and crusting. Biopsy showed subepidermal blistering with a prominent neutrophilic infiltrate. Direct immunofluorescence (DIF) showed positive granular immunoglobulin (Ig)G deposition with C3 and C1q at the dermoepidermal junction which was consistent with bullous SLE (BSLE). The lesions responded dramatically to dapsone 100 mg daily.