An Unusual Case of Pancytopenia: The Lessons Learnt.

Pancytopenia is a common hematological abnormality encountered in clinical practice. We here report a 36-year-old male who presented to emergency department with complaints of weakness of bilateral lower limbs, burning sensation in all four limbs with history of loose stools, and vomiting 5 days back. The complete blood count of patient showed pancytopenia with no circulating atypical cells. Bone marrow examination performed showed nonspecific but characteristic findings. After excluding the possibility of infective etiology, a possibility of heavy metal toxicity was suspected in multidisciplinary meeting. The urine and blood levels of arsenic done came out very high, and a diagnosis of arsenic poisoning was made. Patient had multisystemic involvement with features characteristic of arsenic poisoning. The present case was a diagnostic challenge in face of nonforthcoming history. This case beautifully highlighted the importance of multidepartmental approach in such cases to arrive at unerring diagnosis and the unique bone marrow findings, although nonspecific were sufficient enough to indicate the possibility of acute insult to the hematopoiesis. How to cite this article: Nair RR, Singh PK, Sharma J, Gambhir I, Khanna S, Jain AK, et al. An Unusual Case of Pancytopenia: The Lessons Learnt. Indian J Crit Care Med 2022;26(1):141-144.

Reversal of Glanzmann thrombasthenia platelet phenotype after imatinib treatment in a pediatric chronic myeloid leukemia patient.

Chronic Myelogenous Leukemia (CML) is a myeloproliferative neoplasm characterized by proliferation of Philadelphia positive clonal pluripotent hematopoietic cells. Bleeding is a rare presentation of CML that can occur due to platelet dysfunction. Both pre-treatment and post-treatment platelet function abnormalities in CML have been described in the literature. We describe a rare case of childhood CML who presented with mucocutateous bleeding manifestations. On laboratory workup, a Glanzmann Thrombasthenia (GT) like platelet phenotype was demonstrated along with confirmation of diagnosis of CML in chronic phase. The acquired nature of platelet function defect was confirmed by demonstrating recovery of platelet antigens glycoprotein IIb/IIIa after achieving complete hematological response with Imatinib. Due to presenting complaint of bleeding diathesis and absence of hepatosplenomegaly, the case was undiagnosed for CML until the patient reported to us. Careful evaluation of complete blood counts, peripheral blood picture and detailed laboratory workup was the window to proper diagnosis and treatment in this case.

Carcinoma of Unknown Primary Presenting as Peripheral Symmetric Gangrene.

Cancer of unknown primary accounts for almost 4-5% of all invasive cancers and consists of tumors from various primary sources with considerable heterogeneity in biology and behaviour. Most of these tumors present with symptoms due to distant metastasis. Histology, immunohistochemistry and molecular profiling is the mainstay for diagnosis. In most cases of adenocarinoma associated with paraneoplastic acral vascular syndrome (PAVS), a site in the lung, ovary or uterus is discernible. Here we report a case of metastatic adenoarcinoma of unknown primary presenting as PAVS, a case which to the best of our knowledge has not been reported in published literature.

Isolated Renal Mucormycosis.

Mucormycosis in humans has been described as early as 1885 in literature. Isolated renal mucormycosis is rare as it has been mainly described in developing countries like India and China. It is rarer still to find this entity in immunocompetent young males without any risk factors. Specific guidelines on the treatment is not yet known but combined surgical and medical therapy is considered the best modality for its management. We describe a young male who presented with bilateral hydroureteronephrosis. He was initially treated as a case renal tuberculosis which is relatively more common in TB endemic country like ours. However when he did not respond to the anti-tuberculosis drug (ATT), a biopsy revealed mucormycosis. He was treated with nephrectomy and liposomal amphotericin B and oral posaconazole. On follow up of 2 years he is healthy and leading his normal life.

Cavitary Lung Lesions in a Difficult-To-Treat Asthma Patient.

We describe an interesting case of severe asthma who was not showing satisfactory response to standard treatment. Investigations revealed him to be suffering from allergic bronchopulmonary aspergillosis (ABPA). After starting systemic steroids he showed marked improvement initially only to have recurrent symptoms within a year. He was investigated further and found to have chronic pulmonary aspergillosis in the form of chronic cavitary pulmonary aspergillosis (CCPA) and aspergilloma as also the presence of selective IgA deficiency.

Pulmonary Cryptococcoma Masquerading as Lung Cancer.

We report a case of pulmonary cryptococcoma, in an adult with recently detected diabetes, mimicking as lung cancer. A 45-year-old gentleman with past history of pulmonary tuberculosis presented with fever, cough with expectoration, pleuritic chest pain and hemoptysis. Chest radiograph and computed tomography revealed right lower lobe mass which significantly enhanced on contrast administration. Ultrasound guided biopsy was done which on histopathological examination showed non-necrotizing granulomas with narrow-based budding yeast cells suggestive of cryptococcosis. Detailed work-up for dissemination of infection was negative. A dramatic response to anti-fungal treatment was observed and the patient is doing fine on follow-up.

Consensus & Evidence-based INOSA Guidelines 2014 (First edition).

Obstructive sleep apnoea (OSA) and obstructive sleep apnoea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences amongst the general public as well as the majority of primary care physcians across India is poor. This necessiated the development of the INdian initiative on Obstructive Sleep Apnoea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health & Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep related symptoms or comorbidities or ≥ 15 such episodes without any sleep related symptoms or comorbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents and high risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography (PSG) is the "gold standard" for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances are indicated for use in patients with mild to moderate OSA who prefer oral appliances to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioural measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Unique Triad of 'Pregnancy, Kala Azar and Hemophagocytic Lymphohistiocytic Syndrome from a Non-Endemic Region'.

India and neighboring Nepal, Bangladesh along with Sudan and Brazil are the four largest foci of visceral leishmaniasis and account for 90% of the world's visceral leishmaniasis (VL) burden, with India being the worst affected. High degree of suspicion is usually based on patient presenting from endemic area with features of pancytopenia hepatosplenomegaly. Hemophagocytic lymphohistiocytic (HLH) syndrome also presents with similar clinical features. Visceral leishmaniasis leading to secondary HLH syndrome is in itself a rare entity while both of these presenting in pregnant patient, to the best knowledge of the authors, is yet to be described in literature.

Disseminated Tuberculosis Masquerading as Metastatic Pancreatic Carcinoma.

Tuberculosis (TB) is highly prevalent in India, but TB of pancreas is rare. It is usually seen in patients with miliary TB and often in immunocompromised host as like in HIV positive patients. Pancreatic TB can present as pancreatic mass, pancreatic abscess or acute or chronic pancreatitis. Pancreatic involvement in tuberculosis can occur via haematological route by contiguity from the adjacent organs. In certain clinical settings, presence of pancreatic mass should alert clinicians regarding possibility of pancreatic TB, especially in endemic areas. With use of appropriate diagnostic tests and proper treatment it is potentially curable.

Primary Sjögren's Syndrome Presenting as Flaccid Quadriparesis.

Primary Sjögren's syndrome is an autoimmune disease presenting classically as keratoconjunctivitis sicca. Renal involvement in Sjögrens's syndrome is less common, and the initial presentation with renal complications without any sicca symptoms is extremely rare. The renal involvement may present with symptoms arising from interstitial nephritis, mainly distal renal tubular acidosis.

An Interesting Case of Bilateral Lung Consolidation.

Organising pneumonia is a histopathological entity characterised by intra-alveolar buds of granulation tissue, intermixed myofibroblasts and connective tissue. Cryptogenic organising pneumonia (COP) is characterised by this particular histopathological pattern, along with typical clinical and imaging features, when no other underlying aetiology is found. COP (previously known as bronchiolitis obliterans organising pneumonia [BOOP]) is one of the rare variants of interstitial pneumonias. This condition is characterised by a rapid clinical and radiological improvement with steroid treatment. Here we are reporting a case of COP in adult female with discussion on approach and basic pathophysiology of this type of pneumonia.

Consensus & evidence-based INOSA Guidelines 2014 (first edition).

Obstructive sleep apnoea (OSA) and obstructive sleep apnoea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences amongst the general public as well as the majority of primary care physcians across India is poor. This necessiated the development of the INdian initiative on Obstructive sleep apnoea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health & Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep related symptoms or co-morbidities or ≥ 15 such episodes without any sleep related symptoms or co-morbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents and high risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography (PSG) is the "gold standard" for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances are indicated for use in patients with mild to moderate OSA who prefer oral appliances to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioural measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Imatinib-induced platelet dysfunction and hypofibrinogenemia in chronic myeloid leukemia.

: We aim to present a case of chronic myeloid leukemia (CML) in chronic phase, in major molecular response for 5 years of treatment with imatinib 400 mg OD. He presented with recurrent melena for one and a half years, requiring 11 U of packed red cell transfusion since then. Various causes of bleeding in CML, such as thrombocytopenia, disease progression related to accelerated phase/blast crisis or imatinib-induced cytopenia were ruled out. His investigations revealed reduced plasma fibrinogen (150 mg/ml; range 200-450 mg/ml). The platelet count, prothrombin time, activated partial thromboplastin time and thrombin time were 314 × 10/l, 13 s (control 13 s), 31 s (control 30 s) and 16 s (control 16 s), respectively. Platelet aggregometry revealed normal platelet aggregation with adenine-di-phosphate, epinephrine and ristocetin, and reduced response with arachidonic acid (30%). Bleeding subsided with transfusion of fresh frozen plasma. Moreover, his medication was changed to nilotinib 300 mg BD. Thereafter, his subsequent repeat investigations were normal. Platelet function defects in CML both pretherapy and on tyrosine kinase inhibitors has been described in the literature. However, concomitant hypofibrinogenemia has rarely been reported.

Consensus and evidence-based Indian initiative on obstructive sleep apnea guidelines 2014 (first edition).

Obstructive sleep apnea (OSA) and obstructive sleep apnea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences among the general public as well as the majority of primary care physicians across India is poor. This necessitated the development of the Indian initiative on obstructive sleep apnea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health and Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep-related symptoms or co-morbidities or ≥15 such episodes without any sleep-related symptoms or co-morbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents, and high-risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers, and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography is the "gold standard" for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances (OA) are indicated for use in patients with mild to moderate OSA who prefer OA to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioral measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Consensus & Evidence-based INOSA Guidelines 2014 (First edition).

Obstructive sleep apnoea (OSA) and obstructive sleep apnoea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences amongst the general public as well as the majority of primary care physcians across India is poor. This necessiated the development of the INdian initiative on Obstructive Sleep Apnoea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health & Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep related symptoms or comorbidities or >15 such episodes without any sleep related symptoms or comorbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents and high risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography (PSG) is the “gold standard” for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances are indicated for use in patients with mild to moderate OSA who prefer oral appliances to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioural measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.