Evaluation of the International Society on Thrombosis & Haemostasis scoring system & its modifications in diagnosis of disseminated intravascular coagulation: A pilot study from southern India

Background & objectives: Diagnosis of disseminated intravascular coagulation (DIC) rests primarily on the clinical profile along with supportive laboratory tests. The International Society on Thrombosis and Haemostasis (ISTH) had proposed a scoring system for the diagnosis of overt DIC. However, fibrinogen values which are supposed to be low are often found to be elevated due to the associated inflammation seen in some cases. Moreover, peripheral smear is known to show schistocytes, which is also not included in the score. This study was done to evaluate ISTH scoring system and its modifications in suspected DIC. Methods: Fifty-six patients were enrolled for the present study of whom; in four, fibrinogen assay could not be done. Modifications in the ISTH scoring with the exclusion of fibrinogen, i.e. modified ISTH (MI) score and subsequent inclusion of schistocytes, i.e. modified ISTH with schistocytes (MIS) score, were used. The modified scores were analyzed for diagnostic accuracy parameters and agreement with ISTH score. Results: Amongst 56 cases, 9/52 (17.3%), 22 (39.3%) and 17 (30.4%) were diagnosed as positive for overt DIC by ISTH, MI and MIS scores and mortality was 33, 22.7 and 17.6 per cent, respectively. The sensitivity, specificity, positive and negative predictive values for the MI score were 100, 74.4, 45 and 100 per cent and for MIS score were 100, 86, 60 and 100 per cent, respectively. The agreement between MI score and MIS score with ISTH score was moderate [?=0.502, 95% confidence interval (CI): 0.272-0.732, P<0.001] and substantial (?=0.681, 95% CI: 0.45-0.91, P<0.001). Interpretation & conclusions: In the present study, the calculated mortality was highest by ISTH score. Best agreement was between MIS score and ISTH score. In a resource-constrained setup where fibrinogen assay and therefore ISTH score is difficult, it is suggested that MIS score can be considered.

Primary extranodal non-Hodgkin lymphoma: A 3-year record-based descriptive study from a tertiary care center in Southern India.

Introduction: Primary extranodal lymphoma (pENL) refers to group of disorders arising from tissues other than lymph nodes and even from sites, which normally do not contain lymphoid tissue. This study was undertaken to ascertain the prevalence, anatomical distribution, and histological subtypes of extranodal non-Hodgkin lymphoma (NHL) from a tertiary care institute in Southern India. Materials and Methods: This was a cross-sectional study conducted in the Department of Pathology over a period of 3-years. Detailed clinical history, routine complete blood count, microbiological status was obtained from the medical records. Hamatoxylin and eosin slides were reviewed and immunohistochemistry was performed using a panel of antibodies depending on the morphology. All cases were classifi ed based upon morphologic and immunophenotypic criteria according to World Health Organization 2008 classifi cation. Results: Primary extranodal NHL constituting 22.6% (68/300) of all NHL and the majority of patients were from higher age group with peak incidence seen in fourth to fi fth decade of life. In the pediatric population, the most common site is ileocaecal region (42.8%) and the most common morphology is lymphoblastic lymphoma (42.8%). Similarly in the adult population head and neck was the most common site constituting 36% and diffuse large B-cell lymphoma is the most common morphological subtype comprising 54% of all extranodal lymphomas. Conclusion: Although the reported incidence of pENL is low in India compared to other parts of the world, the possibility of pENL should always be kept in mind even though it arises in an extranodal site.

Spectrum of histopathologic diagnosis of lymph node biopsies: A descriptive study from a tertiary care center in South India over 5½ years.

Aims: Lymphadenopathy is a common clinical problem and biopsies undertaken to determine the cause of nodal enlargement may be neoplastic or nonneoplastic. The former are mainly lymphohematogenous malignancies and metastases while the causes of non-neoplastic lymphadenopathy are varied. This study was undertaken to determine the histopathological spectrum of lymphadenectomies. Materials and Methods: This was a descriptive crosssectional study wherein 1010 cases of histologically diagnosed peripheral lymph node biopsies in the Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry from January 2007 to June 2012 were reviewed. Surgical resection specimens with lymph node dissection were excluded from the study. Results: Neoplastic lesions were more common comprising 53% (535 cases) and included 32.1% (324 cases) of non-Hodgkin lymphoma, 12.4% (125 cases) of Hodgkin lymphoma and 8.5% (86 cases) of metastatic lesions. The non-neoplastic lesions were 47% (475 cases), which included 21.6% (218 cases) of non-specifi c reactive lymphoid hyperplasia, 6.8% (69 cases) of other reactive or specifi c lymphoid hyperplasia, 18% (182 cases) of tuberculous lymphadenitis, 0.6% (6 cases) of other granulomatous lesions. Conclusions: Lymph node biopsy plays an important role in establishing the cause of lymphadenopathy. Among the biopsied nodes, lymphomas were the most common (44.5%) followed by non-specifi c reactive hyperplasia (21.6%), tuberculous lymphadenitis (18%) and metastasis (8.5%).

Nodal mantle cell lymphoma: A descriptive study from a tertiary care center in South India.

Introduction: Mantle cell lymphoma (MCL) is a type of B-cell non-Hodgkin lymphoma (NHL) with distinctive morphologic, immunophenotypic and a characteristic cytogenetic abnormality, the t(11;14)(q13;q32) and overexpression of cyclin D1. The common histologic features include effaced lymphoid architecture by a monomorphic lymphoid population with a vaguely nodular, diffuse or mantle zone growth pattern. The classic cytomorphologic features include small to medium sized lymphoid cells with irregular nuclear contours and scanty cytoplasm, closely resembling centrocytes. Materials and Methods: This retrospective study comprises 13 cases of MCL over a period of 5½ years in our department, comprising 4% of all nodal NHL diagnosed. All cases were diagnosed on lymph node biopsy. Results: The mean age of the presentation was 57 years. There was a male preponderance (M:F = 2.25:1). The disease was nodal in all cases. Most patients (84.5%) had generalized lymphadenopathy and/or hepatosplenomegaly. Bone marrow involvement was seen in 81.8% of cases. Three cases showed a nodular pattern on lymph node biopsy while remaining ten had a diffuse pattern. Immunophenotyping showed positivity for CD20, CD5 and cyclin D1 and CD23 negativity. Conclusion: Despite certain morphological similarity to other low-grade/intermediate-grade lymphomas, MCL has a characteristic appearance of its own. Since it is more aggressive than other low-grade lymphomas it needs to be accurately diagnosed.

A fatal case of bone marrow embolism of unknown cause masquerading clinically as dengue shock syndrome.

Bone marrow fat embolism usually occurs following multiple bone fractures, intraosseous surgical procedures, following vigorous cardiac resuscitation, ecclampsia, sickle cell anemia, malignancies, etc. We present a case of 70-year-old male who presented with fever, cough with expectoration, respiratory distress, altered sensorium, hypotension and thrombocytopenia, and diagnosed to have dengue shock syndrome and expired within 1 day of admission. Postmortem lung biopsy revealed bone marrow fat embolism.

Clinico-hematological profile of Chediak-Higashi syndrome: Experience from a tertiary care center in south India.

Introduction: Chediak-Higashi syndrome (CHS) is a rare autosomal recessive disorder characterized by partial ocular and cutaneous albinism, increased susceptibility to pyogenic infections, the presence of large lysosomal-like organelles in most granule-containing cells and a bleeding tendency. The abnormal granules are most readily seen in blood and marrow leukocytes, especially granulocytes; and in melanocytes. Other clinical features include silvery hair, photophobia, horizontal and rotatory nystagmus and hepatosplenomegaly. Materials and Methods: The clinico-hematological profile of a series of 5 cases of CHS encountered at JIPMER Hospital with diagnostic work-up done in the Department of Pathology over the last 6 years is presented. The diagnostic work-up included complete hemogram with peripheral smear, bone marrow examination, skin and liver biopsies. Results: The age of the patients ranged from 5 months to 3 years. All patients had silvery hair and partial albinism and presented with fever and recurrent chest infection. Two patients were stable. Three patients were in accelerated phase; of them, 1 patient with associated hemophagocytic syndrome had a rapidly fulminant course. Peripheral blood smear showed anomalously large granules in the leukocytes. Skin biopsy showed sparse, coarse melanin pigment in the epidermis, and liver biopsy done in 2 patients with accelerated phase showed portal lymphohistiocytic aggregates. Conclusions: The diagnostic hallmark of CHS is the occurrence of giant inclusion bodies (granules) in the peripheral leukocyte and their bone marrow precursors. The case series is being presented because of the rarity of CHS and varied spectrum of clinical and hematological presentation.

Spectrum of haemoglobinopathies diagnosed by cation exchange-HPLC & modulating effects of nutritional deficiency anaemias from north India.

Background & objectives: The usefulness of cation exchange high performance liquid chromatography (CE-HPLC) as a tool for detection of thalassaemia/haemoglobin variants was evaluated in a prospective study in a tertiary care centre in north India. We also tried to evaluate the effect of concurrent nutritional deficiency on the HPLC pattern in the local ethnic population. Methods: A total of 800 blood samples were analyzed on the Bio-Rad Variant HPLC system by β-thal short program. The retention times, proportion of the haemoglobin (%), and the peak characteristics for all haemoglobin fractions were recorded. Alkaline and acid haemoglobin electrophoresis was performed to document the identities of the haemoglobin variants, wherever necessary. Many cases were subjected to family studies for a definitive diagnosis. Results: Among 800 samples tested, 553 (69.1%) were found to have normal HPLC pattern. Apart from β- thalassaemia, nine additional variants were encountered; HbS (2.8%), HbE (2.5%) and HbD (1.1%) being the most common variants present. Other variants included Hb Q-India, Hb-Lepore, δβ-thalassemia/ HPFH, HbD-Iran, HbJ-Meerut and HbH disease. There was a significant decrease in the level of HbA2 associated with iron deficiency anaemia (IDA) (P=0.004) and increase in megaloblastic anaemia (P<0.001) among subjects with normal HPLC pattern. Interpretation & conclusions: HPLC was found to be a simple, rapid and reliable method for the detection of hemoglobin variants. An accurate diagnosis can be provided in majority of cases by use of retention time, proportion of total haemoglobin, and peak characteristics of HPLC. Haemoglobin electrophoresis and family studies play a valuable role in difficult cases. Concurrent nutritional deficiency also has an effect on HbA2 levels.

Congenital sideroblastic anemia: A report of two cases.

Sideroblastic anemia, comprising of acquired and congenital forms, is a heterogeneous group of disorders characterized by the presence of ring sideroblasts in the bone marrow. Congenital sideroblastic anemia is a rare condition which is mostly X-linked, caused by mutations of delta-aminolevulinic acid synthase 2. We describe two cases of congenital sideroblastic anemia, one of them indicating an autosomal recessive inheritance, with their clinico-hematological profile. It is important to recognize this entity early in life as a significant percentage of cases respond to pyridoxine thus avoiding any long-term complications.

Pseudo Chediak-Higashi anomaly in acute myelomonocytic leukemia.

Pseudo Chediak-Higashi anomaly in acute leukemia is a rarely described entity. The significance of this intriguing morphological finding largely remains unknown, although some authors have predicted a poorer outcome in such cases because of a higher susceptibility to fulminant infections. Our case also had a fatal outcome.

Clinically unsuspected Hodgkin's lymphoma diagnosed primarily from bone marrow trephine biopsy: report of six cases.

Bone marrow may be the initial or rarely the only site of involvement in Hodgkin's lymphoma. A high index of suspicion is required to pick up the histopathological changes of Hodgkin's lesions in the bone marrow like necrosis, presence of Reed-Sternberg cell or its variant in a polymorphic background infiltrate, focal fibrosis and myxoid change especially in the absence of classical clinical picture. Bone marrow with immunohistochemistry has a valuable role in the staging and in the diagnosis of primary medullary Hodgkin's lymphoma. B-symptoms may easily masquerade as an infectious process as in all our cases the patients had fever as a presenting feature, in four of them tuberculosis was suspected clinically and two had received antitubercular therapy elsewhere. We report six human immunodeficiency virus-negative patients diagnosed over a period of 5 years in which the initial diagnosis of Hodgkin's lymphoma was suggested from bone marrow histology.

Cryptococcal neoformans profiles in peripheral blood neutrophils: an unusual presentation.

We report an unusual observation of Cryptococcal neoformans profiles engulfed by neutrophils on a routine peripheral blood smear examination in an HIV-negative young female patient who presented with perforation of large bowel following a pregnancy termination procedure by an untrained midwife.

A case of rhabdomyosarcoma masquerading as acute leukemia at presentation: a case report.

Non-hematopoietic malignancies infiltrating bone marrow have always been a source of erroneous diagnosis. Among these, the small round cell tumors like neuroblastomas and rhabdomyosarcomas mimick the hematopoietic blasts. Several case reports of rhabdomyosarcoma mimicking acute leukemia, clinically and morphologically at presentation have been reported in the literature. To the best of our knowledge such an entity has not been reported in Indian literature. We report here one such case of alveolar rhabdomyosarcoma masquerading as acute leukemia. A thorough clinical examination with high degree of suspicion on bone marrow morphology and judicious use of appropriate immunohistochemistry markers will solve many of these cases.