A 67-year-old female was admitted to our emergency in altered sensorium with generalized erythroderma and patchy hair loss. She underwent splenectomy for lymphangioma [Table/ Fig-1] six months before. Clinical examination showed erythroderma, sacral edema, mucosal dark pigmented lesions, patchy hair loss and madarosis. Basic laboratory investigations showed anaemia (Haemoglobin-7.3 gm/dl), leucocytosis (White blood cell count-19,000/c.mm) with hypereosinophilia (Absolute eosinophil count : 11,768/c.mm) and thrombocytosis (5,08,000/c.mm).She had severe hypoalbuminemia (albumin -1.9 gm/dl) probably secondary to loss of protein through skin. Anti-nuclear antibody (ANA), anti-double stranded DNA (anti-Ds DNA) and antineutrophil cytoplasmic antibody (ANCA) were negative. In the absence of any significant history of atopy, allergic disorders, or parasites to explain her high eosinophil counts she was submitted to a work up for primary hypereosinophilia and any associated end organ damage. In view of high Vitamin B12 levels (16,680ng/L) and hypereosinophilia, there was a strong suspicion of myeloproliferative disorders. Molecular genetic work up showed absence of Fip1-like-1 fused with platelet derived growth factor receptor alpha (FIP1L1-PDGFRα) and BCR-ABL mutation. Bone marrow biopsy showed scanty marrow with eosinophilia [Table/ Fig-2].T-cell receptor rearrangement studies were done and primary cutaneous T cell lymphoma was ruled out. A skin biopsy showed subcorneal eosinophilic collection with spongiotic dermatitis [ Hypereosinophilic syndrome (HES) is a myeloproliferative disorder characterised by marked peripheral eosinophilia and end organ damage attributable to eosinophilia without secondary cause. Early recognition and treatment are essential to prevent morbidity and mortality. Cytoreduction with Steroids, Hydroxyurea and Imatinib are the main stay of treatment. Molecular studies like Fip1-like-1 fused with platelet derived growth factor receptor alpha (FIP1L1-PDGFRα) etc., are recommended in view of therapeutic implication. In this paper we report a rare case of HES developing in a lady 6 months after surgical removal of lymphangioma of spleen, which in itself is rare.

Objective: Multiple myeloma (MM) is a clinically and genetically heterogeneous plasma cell neoplasm. The prognosis of MM patients is dependent on several factors including the patient’s age, the stage of disease and genetic alterations. This study aimed to determine the frequency of common chromosomal abnormalities and their significance in MM patients referred to a tertiary healthcare center in India. Methods: Fluorescence in situ hybridization on interphase nuclei from bone marrow cells using seven MM-specific probes for recurrent aberrations was performed in a total of 215 newly diagnosed patients. Results: Chromosomal abnormalities were detected in 161 (74.9%) MM patients in this study. The most frequent aberration was trisomy(ies) involving only gain of chromosomes in 48 (22.3%) cases. A translocation involving the IGH gene alone or accompanied by trisomy(ies) or by monosomy 13/13q deletion or by both was registered in 80 (37.2%) patients. Atypical patterns such as a deletion of the IGH variable segment (IGHv) on the derivative chromosome 14 or on the native (normal) chromosome 14, biallelic deletion of IGHv, deletion of the IGH constant segment on the rearranged chromosome14 and extra fusions were noticed in 21 (9.8%) patients with an IGH rearrangement. Monosomy 13/deletion 13q was identified singly or as part of a complex karyotype in 74 patients (34.4%). Clonal heterogeneity and additional abnormalities including TP53 deletion and monosomies of chromosomes 4, 9, 14 and 16 were recorded in 18.6% and 16.3% of patients respectively. Patients with abnormalities exhibited plasmacytosis, reduced hemoglobin value and high level of ß2-microglobulin. Conclusions: A lower median age and a low frequency of IGH translocations particularly t(11;14) and chromosome 13 abnormalities suggest ethnic diversity. Further investigations on genetic alterations including IGH deletions will contribute to improved insights into the biology of myeloma disease, risk stratification and patient management.

Plasma cell myeloma (PCM) is a hematologic malignancy of plasma cell origin and usually associated with the presence of lytic bone lesions. Pleural effusions are rarely associated with PCM and most often signify a concurrent disease process. Malignant myelomatous pleural effusions are even more unusual and carry a poor prognosis. We report a unique case of unsuspected PCM with thoracic involvement in the form of massive left side pleural effusion. Pleural fluid cytology revealed numerous atypical plasma cells. Subsequently on further workup, urine Bence Jones protein was positive. Bone marrow aspiration and biopsy and computed tomography of the chest and abdomen revealed features consistent with multiple myeloma.

Amyloid Light chain (AL) amyloidosis is a rare disease, which is seen in approximately one-tenth of patients with multiple myeloma. We report a 52 years old male, who presented with complaints of anorexia and weight loss. He was diagnosed to have multiple myeloma-international staging score (ISS) Stage 3 and was started on VTD (Bortezomib, Thalidomide, and Dexamethasone) chemotherapy. Within 2 weeks of therapy, he had abdominal symptoms like abdominal pain, loose stools, vomiting and hematochezia. Imaging showed dilated proximal bowel loops with fluid filled contents and prominent vessels in rectum. Emergency surgical exploration revealed thickened proximal jejunum with blood clots in the lumen. Resection of proximal jejunum was done. Histopathological examination of resected specimen was suggestive of AL amyloidosis. Post-surgical resection of jejunum, patient had initial improvement followed by deterioration. He was discharged against medical advice as per relative’s request. Hence an index of clinical suspicion of amyloidosis must been present in all Multiple myeloma patients.

No abstract