Nilesh Kumar

Unilateral RS3PE in a Patient of Seronegative Rheumatoid Arthritis.

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare but well-reported clinical entity. It is classically described as symmetrical involvement of both upper extremities. Asymmetrical involvement had also been reported, but unilateral presentation is very rare. We hereby report a case of unilateral RS3PE in a patient of seronegative rheumatoid arthritis which was initially misdiagnosed as cellulitis and was given high dose antibiotics without any significant improvement. Later a rheumatologic consultation leads to a prompt diagnosis, and treatment with steroids leads to dramatic reversal of symptoms. This case demonstrates the rare presentation of this rare clinical entity and highlights the necessity of awareness regarding unilateral disease to clinicians.

Clinical profile of chronic daily headache in patient attending tertiary centre

Background: The frequent headache syndrome is among the most challenging disorder that conforms clinician on a routine basis. A major problem with the relatively new form chronic daily headache (CDH) has been its confusion with chronic tension type headache (CTTH) and other community used term for which there have never been operational diagnosable criteria or even informal clinical criteria. The medical literature is replete with example over the past three decades in which the terms mixed headache combined headache tension headache; vascular headache and rebound headache are used interchangeably by interference. CDH became synonymous in many clinical setting with CTTH or inconvenient vascular or migrainous features or medication overuse. Now CDH is well described in literature with defined classification. Hence, the researcher is used to interested what is the burden and proportion of the various type of CDH. Aims and Objectives: Primary aim of this exploratory prospective study was to study the incidence of primary headache and secondary headache which were CDH in patient attending neurology outpatient department, to study the incidence of type of primary headache, the cause of secondary headache, the age wise distribution and sex wise attribution of CDH and to study the impact of family history, occupation and precipitating factor on CDH. Materials and Methods: In our study, a total of 626 cases fulfilling the International Headache Society-4th revised criteria comprising inclusion and exclusion criteria were included after informed consent, meticulous history taking pertaining to headache, in particular, thorough examinations and relevant laboratory investigations, and were certified by one neurologist. Results: Primary headache including migraine, tension-type headache, Short-lasting unilateral neuralgiform headache with conjunctival injection, new daily persistent headache, and medication overuse headache was the most common headache that caused CDH. Neurocysticercosis was leading cause of a secondary headache that leads to CDH. Light, fast, and stress were leading precipitants associated with CDH. Conclusion: CDH was mostly associated with primary headache over the secondary headache. Chronic migraine was leading cause of CDH in comparison to CTTH. Females were more affected both in chronic migraine and CTTH. Family history was more positive in case of chronic migraine. Vomiting nausea, photophobia, and phonophobia vertigo were leading symptoms associated with CDH whereas light, fast, and stress were leading precipitants associated with CDH.

Distribution of ABO and rh blood group in myeloproliferative diseases

Background: The aim of this study was to investigate the distribution of ABO and Rh blood group in myeloproliferative disease and if association, if any exists can be used as a preclinical marker for cancer development and risk; hence, we can modify other modifiable risks for myeloproliferative diseases (MPDs). Materials and Methods: This was a cross-sectional study which included 252 cases who were diagnosed as a patient with lymphoid malignancies of any age group of both gender, 350 controls were taken as healthy people who have donated blood. Cases and controls were collected between January 1, 2016 and May 30, 2017. Blood group sampling of all cases and controls was performed. The obtained information regarding the presence or absence of myeloproliferative disorder, blood group type, age group, and type of myeloproliferative disorder analyzed, the statistical analysis was performed using SPSS for windows version 16.0 software. The findings were present in number and percentage analyzed using frequency, percentage, and Chi-squared test. Results: Of 266 cases of MPDs, majority of cases were of blood Group B (35.34%), followed by O (27.81%), A (26.69%), and AB (10.16%). Majority of cases were Rh positive (85.33%). Of 266 cases of MPDs, the most common was chronic myeloid leukemia (CML) (53.38%), polycythemia vera (16.55%), essential thrombocytosis (15.78%), primary myelofibrosis (11.28%), mastocytosis (1.5%), chronic neutrophil leukemia (1.13%), and the least common chronic eosinophilia leukemia (0.38%). In CML, primary myelofibrosis, mastocytosis, chronic neutrophilic leukemia, and chronic eosinophilic leukemia blood Group B was the most common, while blood Group O was more common in polycythemia vera and essential thrombocytosis. In control group, the most common blood group was O (36%), followed by B (32.67%), A (21.33%), and AB (10%), respectively. Majority of control groups (94.33%) were Rh positive and (5.67%) were Rh negative. Conclusions: The present study revealed that there are significant associations between ABO blood group and patients with MPDs. Male gender and blood group B and Rh factor positive are more prone for the development of myeloproliferative disorder. These findings also raise the possibility of using blood groups as an epidemiological marker for identifying population subgroups who are at high risk of these MPDs.

Study of possible association of chronic typhoid infection in patients with chronic leukemia

Objective: The study of the possible association of chronic typhoid infection in patients with chronic leukemia. Materials and Methods: A total of twenty patients with chronic myeloid leukemia (CML) either newly diagnosed or follow-up patients with or without chemotherapy in the chronic phase of the disease and ten patients with chronic lymphocytic leukemia had comprised of the study. Bone marrow (BM) samples of 20 controls had been taken from the Orthopedic department during surgery. BM was aspirated from iliac crest under aseptic precautions and BM biopsy was done, samples were stored at 4°C. BM samples were subjected for polymerase chain reaction (PCR) analysis for Salmonella typhi. Blood and stool sample from the patients were also collected for Salmonella culture, serological assays. BM samples from control had been taken during orthopedic surgery. Results: S. typhi infection, the comparison between CML patients and healthy controls had been made based on the PCR results. The result is significant with 40% positivity for S. typhi PCR in CML patients when compared to 20% positivity in healthy controls with P = 0.028 which is statistically significant. In patients with chronic lymphocytic leukemia, 10% of BM samples showed positivity for S. typhi by PCR targeting flagellin gene-specific nucleotide sequences and 10% of controls showed positivity for S. typhi and was statistically insignificant (P = 1.00). Conclusion: The early incidence of the disease in tropical country like India favors the hypothesis of chronic inflammation in the early process of leukemogenesis. The result of the study also questions whether attenuated bacteria can be used safely for vaccination and delivering therapeutic agents. Moreover, in a tropical country like India where S. typhi infection is endemic, the infection with the same can be taken as a marker of chronic inflammation and its role in the etiopathogenesis of CML remains to be understood.

Clinicomicrobiological profile of the Indian elderly with sepsis

The study included 400 elderly patients (≥60 years of age) with clinically suspected sepsis. The maximum number of patients was in the age group of 60-69. Diabetes was the most prevalent comorbid condition. The most common site of infection was the urinary tract followed by the lungs atypical manifestations were quite prevalent with delirium being present in majority of patients. The most common organism isolated was Staphylococcus aureus followed by Escherichia coli. Introduction: Sepsis is one of the leading causes of morbidity and mortality in the elderly; despite great progress in antimicrobial treatment and intensive care medicine, the incidence of sepsis remains high while severe sepsis still has high mortality. Materials and Methods: The study included 400 consecutive elderly patients (≥60 years of age) with clinically suspected sepsis. Sepsis definition was based on the presence of infection and two or more of the systemic inflammatory response syndrome (SIRS) criteria. Blood and other site cultures were obtained on admission and during hospitalization when needed. Result: The total number of elderly patients with suspected septicemia who were enrolled in the study was 400. The maximum number of patients (68%) were in the age group of 60-69 years, whereas 32 patients (8%) were in the age group of >80 years. The age of the study population ranges from 60 years to a maximum of 95 years. The mean age of our study population was 67.52 ± 6.65 years with a male: female ratio of 1.68. The prevalence of SIRS criterion in the study population shows that tachycardia (89%) was the most common criteria followed by fever (84%) and leukocytosis (82.5%). All the four SIRS criteria were present in 42% of the patients, three in 37.5% of the patients and only two in 20.5% of the patients. The most common site of infection was the urinary tract (30.5%) followed by the lungs (21.25%) and skin infection (14%). Atypical manifestations were quite prevalent in the study population with delirium being the most common presentation. Conclusion: The present study was undertaken to study the clinicomicrobiological profile in Indian elderly patients presenting with sepsis. The mean age of the study population was 67.52 ± 6.65 years. Out of 400 cases, there were 115 (28.75%) blood culture positive cases. Gram-negative organisms (51.7%) were more commonly grown than gram-positive ones (48.30%). The most common organism isolated was Staphylococcus aureus (49 patients) followed by E. coli (36 patients). Diabetes was the most prevalent comorbid condition in our study population that was present in 28% of the population followed by BPH (17.75%) and hypertension (16%). Delirium was present in 112 (28%) patients not having CNS infection. In our study, 324 patients improved, whereas 76 died and the mortality was positively correlated with the stage of sepsis and it was statistically significant (P = 0.032).

Paraparesis in Coarctation of Aorta: Diagnosis in Disguise.

To the Editor: A 55-year-old woman presented with sudden onset symmetric weakness of both lower limbs along with truncal weakness and urinary retention for 7 days. However, she denied any history of pain, trauma, loss of consciousness, or any evidence of upper limb weakness, breathlessness, or cranial nerve involvement. According to history, a provisional diagnosis of acute transverse myelitis was made, for which she was being treated before coming to the Institute of Medical Sciences, Banaras Hindu University. On examination, the pulse of lower limbs was feeble as compared to upper limb pulse. Blood pressure of upper limb was 210/100 mmHg, while it was not recordable in the lower limbs. Prominent superficial blood vessels were visible on her back in the interscapular region, and bruits could be palpated along her intercostal spaces. Neurological examination revealed complete loss of power (grade 0/5) in lower limbs with hypoesthesia below C7 vertebra. Deep tendon reflexes were absent, and plantar reflexes were not elicitable. Examination of the upper limbs and cranial nerves were normal. There was no spinal tenderness or deformity. Systolic murmur prominent at aortic area was noted on auscultation. The rest of the systemic examination was within normal limit. Basic blood investigation including hemocoagulation tests was within normal limit. A chest X-ray showed notching of inferior margins of ribs with a normal cardiac shadow and lung parenchyma. Electrocardiograph revealed sinus rhythm with left ventricular enlargement. Two-dimensional echocardiography showed the presence of moderate left ventricular hypertrophy with decreased flow in the descending thoracic aorta beyond the origin of left subclavian artery, with no other valvular or congenital cardiac malformations. Computed tomography of aorta revealed a circumferential constriction of the descending aorta of length 1.5 mm and distance 2.1 cm from the origin of left subclavian artery, with 82% stenosis of the lumen, which suggested that the patient had coarctation of aorta (CoA). A large number of intercostal and phrenic collaterals were also seen, along with tortuous bilateral internal mammary arteries and inferior epigastric arteries. A contrast enhanced magnetic resonance imaging of the spine showed the presence of multiple tortuous engorged vessels in epidural and intradural spaces along mixed signal intensity lesion with convex posterior margin noted in epidural space of C7, D1, and D2 suggestive of epidural hematoma at C7–D2 level adhered to the dura causing spinal cord compression [Figure 1]. This compression of the spine cord was severe enough to cause myelopathy below the level of C7 in the form of sudden onset of flaccid weakness of truncal and lower limb muscles due to spinal shock. Because of the limited options of surgical management at our center, we referred the patient to higher center for further treatment. Figure 1 Multiple tortuous engorged vessels in epidural and intradural spaces along with an epidural hematoma at C7–D2 level adhered to the dura causing cord compression (a-c). CoA has an incidence of 0.3–0.4/1000 live births and accounts for 5–7% of adults with congenital heart disease. Usually associated complications with it are aortic dissection, infective endocarditis, and rupture of aneurysms in the circle of Willis leading to intracranial hemorrhage. Spinal cord complications of CoA are rare, and only a few cases have been reported due to subarachnoid hemorrhage following rupture of an aneurysm of abnormally dilated spinal artery.[1,2] Compressive myelopathy due to collaterals in the spinal cord is a very unusual presentation, and few reports have been reported worldwide.[3] The development of an acute, nontraumatic epidural hematoma is even rarer.[4] Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Lymphoma without Lymphadenopathy

A seventy-five years old man was presented to our hospital with a complaint of gradually progressive heaviness in the left half of abdomen for past 10 months. History was negative for fever, weight loss, and night sweats. Physical examination was noteworthy for moderate pallor and massive splenomegaly [Figure 1a]. Figure 1 Splenic area marked (a); Peripheral blood film (b); Ultrasonography showing splenomegaly (c). There was no lymphadenopathy. Routine investigations showed: Hemoglobin 70 g/L, total leucocyte count: 98.4 × 109/L, neutrophils: 4%, lymphocytes: 93%, monocytes: 2%, and platelet count: 103 × 109/L. Peripheral blood smear showed markedly increased the number of leukocytes with a fair number of smudged cells. Red blood cells showed anisopoikilocytosis with the predominantly normocytic normochromic picture [Figure 1b]. Platelets were reduced in number to 103 × 109/L. Renal and liver function tests, serum lactate dehydrogenase (298 U/L), serum uric acid levels (6.5 mg/dl), chest X-ray, and electrocardiogram were within normal limits. Coombs test was negative, and stool analysis was negative for occult blood. The ultrasonography of abdomen showed gross splenomegaly [Figure 1c]. Contrast-enhanced computed tomography (CT) of chest and abdomen confirmed splenomegaly and did not reveal additional information. However, positron emission tomography-CT imaging was not performed. Conventional karyotyping was normal. The patient did not give consent for splenic aspiration. Bone marrow aspiration and biopsy revealed diffuse infiltrates of small lymphoid cells with nuclear round contour and scanty cytoplasm, suggestive of chronic lymphocytic leukemia (CLL) [Figure 2a]. Immunophenotyping by flow cytometry revealed: CD79b (77.1%) was strongly positive, CD5 (98.1% of gated lymphocytes), and CD23 (51.1%) were positive. FMC-7 was positive (51.1%), and surface immunoglobulin (1.7%) was negative. Immunophenotyping results were disfavoring CLL/small lymphocytic lymphoma (CLL/SLL) as according to the scoring system, a score of 4 or 5 is required for the diagnosis of CLL, whereas here the score was 3. Figure 2 Bone marrow trephine biopsy (a); Fluorescence in situ hybridization was done, and it was positive for IgH/CCND1 fusion signal (b) Typically, CLL cells express weak monotypic surface immunoglobulin, CD5, CD19, CD23 and weak or absent CD79B, CD22, and FMC7. Using a recommended panel of scoring system for the diagnosis of CLL.[1] Ninety-two percent of CLL cases score 4 or 5, 6% score 3, and 2% score 1 or 2. Most other chronic B-cell lymphomas and leukemias score 1 or 2, but a minority score 3. Scores in CLL are usually >3, in other B-cell malignancies the scores are usually <3. The scatter parameters and antigen expression profile as studied by flow cytometry of the sample were suggestive of atypical chronic lymphocytic leukemia, B-CLL. No single type of lymphoma/leukemia was matching with clinical and flow cytmetry parameters. In spite of the absence of lymphadenopathy, presence of massive splenomegaly and lack of “CD23 negativity” mantle cell lymphoma (MCL) was a possibility before labeling it as atypical B-cell CLL, as there are case reports of MCL without lymphadenopathy and with CD23 positivity, but the combination of all three parameters (absence of lymphadenopathy, presence of massive splenomegaly, and CD23 positivity) were making possibility of MCL dubious. But flow cytometry results prompted us to pursue the diagnosis of MCL as it was the only other plausibility apart from CLL, for which flow results were least unfavorable. Fluorescence in situ hybridization (FISH) was done, and it was positive for IgH/CCND1 fusion signal. IgH/CCND1 fusion signal was detected in 92% cells by FISH [Figure 2b]. MCL is one of the several subtypes of B-cell non-Hodgkin lymphoma. The distinction between SLL/CLL and MCL has important clinical implications. Typically, SLL/CLL is CD23+, whereas MCL is CD23−. The majority of MCL cases co-express CD20, CD5, BCL2, and cyclin D1. MCL is usually negative for CD10, BCL6 and CD23. Chromosomal translocation characteristic for MCL is t(11;14)(q13;q32), which results in overexpression of the cell cycle protein cyclin D1.[2] In reports that include CD23+ MCL cases, detected by flow cytometric or immunohistochemical analysis, the frequency of CD23 expression has ranged from 2% to 45%. MCL is an aggressive lymphoma with moderate chemosensitivity. Reliably curative treatments for MCL are lacking. An inexorable pattern of progression is characteristic, with a median time to treatment failure of <18 months.[3,4] The absence of lymphadenopathy and presence of CD23 positivity makes this case a rare one among all MCL cases. This patient was started on bendamustine rituximab chemoregimen for 6 cycles, and he showed partial remission after completion of the forth cycle. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Acute-onset paralysis in a patient of rheumatoid arthritis.

Renal tubular acidosis (RTA) is a disorder of renal acidification characterized by inability to acidify urine to pH <5.5 despite the presence of severe systemic metabolic acidosis and hypokalemia. Hypokalemia leads to acute-onset paralysis and may be a presenting manifestation of RTA. Its association with various autoimmune disease has been reported previously in published reports, but has not been much emphasized. We, hereby, report a case of RTA that presented during the flare of rheumatoid arthritis (RA). A 42-year-old female, a known case of RA for 5 years, presented with persistent joint pain for 1 week and acute-onset quadriparesis for 3 days. Primary investigations revealed hypokalemia with metabolic acidosis. She was managed conservatively with potassium supplements and bicarbonate supplements along with steroids and disease-modifying anti-rheumatic drugs. Such a presentation of renal tubular acidosis in a patient during the flare of rheumatoid arthritis is distinctly rare and previously unreported in published studies.

Forestier’s disease with complete spinal involvement

A 70-year-old diabetic and hypertensive male patient visited our clinic with complaints of non-inflammatory back pain since two years. He was heavy built with a BMI of 29 kg/m 2 . Physical examination revealed limitation of flexion, extension, and lateral flexion of lumbar spine. X-ray of spine revealed flowing calcification of anterior longitudinal ligaments from cervical to lumbar spine with claw osteophytes giving rise to characteristic ‘Melting Wax’ appearance (Panel A: fig A- cervical anteroposterior (AP), fig B- cervical lateral , fig C- lumbar AP , fig D- lumbar lateral) (Panel B: fig E- thoracolumbar AP, fig F- thoracolumbar lateral). Sacroiliac joints were normal (Panel A: fig C- white arrow). The diagnosis was diffuse idiopathic skeletal hyperostosis (DISH or Forestier’s disease) and symptomatic treatment was initiated.

Congenital block vertebrae in lumbar spine

A 34-year-old female visited our rheumatology clinic with the complaint of non-inflammatory back pain that usually occurred on bending down and radiated to bilateral lower limbs. A diagnosis of compressive neuropathy was suspected and an initially performed digital X-ray of lumbar spine revealed a rare congenital anomaly termed as ‘Congenital block vertebrae’ in the lumbar spine (Fig 1). Block vertebrae, a congenital anomaly, occurs due to improper segmentation of vertebral column during fetal development. Improper segmentation leads to fusion of adjacent vertebrae through their inter-vertebral disc. The most common site of this deformity is cervical spine and the lumbar vertebral involvement is rare. The disco-vertebral articulation is always involved and depending on the degree of involvement, the presenting complaints could be either neurological due to the compression of nerve roots or scoliosis due to the vertebral deformity.