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Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia with or without bleeding. Mild to moderate thrombocytopenia can be seen with thyrotoxicosis and hypothyroidism. This study was conducted to assess prevalence of thyroid dysfunction in ITP and impact on treatment and outcomes. This prospective study included patients more than 12 years old, diagnosed as ITP. Serum Thyroxine (T4) and Thyroid Stimulating Hormone levels and anti-thyroid peroxidase (TPO) antibodies were done by electro- chemiluminescence immunoassay. A total of 168 patients were enrolled, with thyroid function tests available in 146 patients. Mean age was 30.6 years, with 67.8% females (n = 114). Sixty percent patients had chronic ITP and 25% had persistent ITP. Overall prevalence of thyroid disease was 25.7% in the study population, with overt hypothyroidism seen in 21 (16.4%) patients, subclinical hypothyroidism in 9 (7.0%) patients and subclinical thyrotoxicosis in 3 (2.3%) patients, while no patient had overt thyrotoxicosis. Thyroid status of the patients did not correlate with duration of ITP or response to treatment. Presence of anti TPO antibodies was associated with abnormal thyroid function and chronic ITP. The prevalence of thyroid dysfunction is increased in ITP patients. Many such cases have anti TPO antibodies, suggesting autoimmune pathology. Role of glucocorticoids, estrogens and systemic illness in these findings must be further studied before firm conclusion can be drawn about their routine inclusion in diagnostic work up.

In COVID 19 pandemic, delivery and access of health care services have become challenging. Telemedicine services can be considered for management of patients with hematological diseases. This study included all patients who enrolled for telemedicine facility for hematology from May 15 to July 15, 2020. Patient’s demographic and disease related parameters were recorded during the teleconsultation call. Overall satisfaction of attending doctor and patients were also recorded. A total of 1187 teleconsultation appointments were taken, of which 944 (79.6%) were successfully attended. Median age of patients was 38 years (range- 0.5–78 years), with 38% females. 55% of successful calls were from patients suffering a malignant hematological disorder. 24% had an active complaint pertaining to their disease or treatment. Of these, 162 (17%) were asked for a physical consultation. A significant association was found between the requirement of physical consultation and diagnosis (p

Acute graft-versus-host disease (aGVHD) and relapse are major issues for patients undergoing allogenic hematopoietic stem cell transplant (allo-HSCT). T-regulatory (Treg) cells in the donor graft are negatively correlated with the incidence of aGVHD without any impact on relapse. In this study to determine the association of Treg cells with aGVHD in allo-HSCT patients. Thirty-two patients with hematological disorders, who underwent allo-HSCT. Twenty-nine patients who achieved engraftment were enrolled in the study. Treg cells were quantified in donor graft by flowcytometry and were assessed for their association with aGVHD and other clinical outcomes. Fifteen of 29 patients developed aGVHD. According to the occurrence and severity of aGVHD, patients were divided into two groups: 20 (68.9%) patients with grade 0–I aGVHD and 9 (31.1%) patients with grade II–IV aGVHD. Treg cells/CD4 ratio was significantly higher in the grade 0–I aGVHD group than in grade II–IV aGVHD group, (p = 0.0002). We could not find the association of CD34 dose (p = 0.55) or CD3 dose (p = 0.57) with the severity of aGVHD. Higher Treg cells/CD4 ratio in donor graft was associated with less severe aGVHD. Though more studies are needed, Treg cells/CD4 ratio may be used as a predictive marker for severity of aGVHD in post allo-HSCT.

To determine the efficacy of zoledronic acid (ZA) in thalassemia major associated low bone mineral density. Prospective, open label, single arm trial. Bone mineral density (BMD) at lumbar, hip and forearm region were performed at baseline and after 1 year of therapy. Initial, 9 patients received a first dose of 4 mg. Due to severe adverse effects, further doses for these patients and all new recruited patients were 1 mg once every 3 months for 4 doses. All patients were receiving 500 mg of calcium carbonate twice daily and 0.25 μg alfacalcidol once daily before and during the entire study period. Dual energy X-ray absoptiometry was performed at baseline and after 1 year. Twenty-seven patients with transfusion dependent thalassemia with a median age 19.5 year (15–38 years) were eligible for ZA treatment. Seven patients had bony pains. Four patients developed grade 4 hypocalcemia (3 developed tetany) and 2 developed infusion related toxicity with initial dose of 4 mg. One mg dose was well tolerated. At the end of 1 year, bone pains had completely resolved. There was significant increase in BMD at lumbar (p = 0.002) and forearm regions (p = 0.04) and intertrochantric area (p = 0.041). The % change in BMD at 1 year was +3.7 ± 3.2%. ZA is an efficacious agent in treatment of low BMD in these patients. ZA produces significant adverse reactions at 4 mg dose but 1 mg dose is well tolerated and is efficacious.

Acute Myeloid Leukemia (AML) as per World Health Organization (WHO 2008) classification is on the basis of the antigenic characterization, enzymes restriction in the neoplastic myeloid cells and the specific translocations/mutations. AML can be assessed and differentiated by flowcytometry (FCM)/immunohistochemistry (IHC)/cytochemistry techniques. Myeloperoxidase (MPO) is an unequivocal marker to differentiate AML from the acute lymphoblastic leukemia. Despite FCM popularity, it has its limitations, in form of ‘dry-tap’, cost, and inability of being performed by retrospective analysis. IHC, though an old technique has overcome these disadvantages of FCM. Cytochemistry, on the other hand has its own advantages in being cost-effective; technically easy to do while its disadvantages are its inability to be carried out in the old samples, ‘dry-tap’ conditions in aleukemic leukemia. There has been non-uniformity in the literature among these techniques especially concerning their sensitivity for MPO. A prospective study was done at All India Institute of Medical Sciences New Delhi from 01 July 2014 to 30 Nov 2015 to include 120 diagnosed acute myeloid leukemia cases. Myeloperoxidase stain was done by cytochemistry, immunohistochemistry and flow cytometry and results were compared. There were 28 cases which showed discrepancies. Out of these 28 cases immunohistochemistry showed positivity in majority (22 cases) followed by flow cytometry (14 cases). Therefore it is important to employ more than one technique and IHC must be included for detection of MPO in all suspected cases of AML especially when negative with FCM .

A comprehensive laboratory diagnosis of hemoglobinopathies forms an integral part in workup of disorders of globin chain synthesis. Clinical findings, complete blood counts, peripheral smear examination along with hemoglobin (Hb) electrophoresis and/or cation exchange high performance liquid chromatography findings and parental study helps to clinch a final diagnosis. Compound heterozygous hemoglobinopathy presents with variable clinical findings and some of them are picked up on screening tests done as part of routine antenatal workup. Here we report a rare double heterozygous hemoglobinopathy of Hb D-Punjab and Hb J-Meerut in a 35 year antenatal female.

Hereditary hypotransferrinemia is a very rare cause of iron deficiency anemia in childhood characterized by microcytic hypochromic anemia refractory to iron therapy and concomitant iron overload. Regular plasma infusion to replace the deficient transferrin molecule is the therapeutic option. We report two cases; both presented with refractory anemia requiring blood transfusions and responded to monthly fresh frozen plasma replacement.

Aplastic anemia (AA) is a life-threatening bone marrow failure disorder, if untreated, is associated with very high mortality. Allogenic bone marrow transplantation (BMT) is the standard of care for severe aplastic anemia (SAA) patients those who are younger than 40 years of age. The development of secondary malignancies in post-BMT setting for AA is a rare, however, well documented phenomenon. Among the secondary malignancies, development of acute myeloid leukemia is even rarer entity. Here we report a case of acute myeloid leukemia following human leucocyte antigen (HLA) matched sibling peripheral blood stem cell transplant (PBSCT) in a case of SAA. The patient achieved complete remission (CR) following chemotherapy and in CR1, a second HLA matched PBSCT from a different donor was offered. The patient is presently in remission at day +180 post-PBSCT.

Forty-five years old male, a known case of T cell acute lymphoblastic leukemia (ALL) on maintenance therapy presented with bilateral painless progressive diminution of vision. Evaluation revealed cytomegalovirus (CMV) retinitis with low CD4 counts. CMV retinitis is usually seen in HIV disease or in post allogenic stem cell transplant recipients. CMV retinitis occurring in ALL maintenance phase is very rare. The disease is aggressive and shows incomplete response to medical therapy.

Pulmonary complications are one of the most common causes of morbidity and mortality in patients undergoing peripheral blood stem cell transplantation. Both infective and non-infective etiologies can involve the lungs during this period and differentiating them clinically is a challenging task and management differs in each case. We present here a case of acute myeloid leukemia, in whom following allogeneic peripheral blood stem cell transplantation, diffuse alveolar hemorrhage developed.

Dear editor, Priapism, that is, persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation, is a relatively uncommon disorder and is a medical emergency. Typically, only the corpora cavernosa are affected [1]. The term priapism was derived from the Greek god Priapus, son of Aphrodite who was born with oversized genitals [2]. The haematological causes for priapism include Sickle cell anaemia, Leukaemia, Multiple myeloma, Paroxysmal nocturnal haemoglobinuria, Thalassaemia, Thrombocythemia and Henoch–Schonlein purpura [3]. As far as hemoglobinopathies are concerned, priapism is most often encountered in sickle cell disease in 38–42 % of cases followed by sickle/beta thalassemia [4, 5]. Priapism is also noted in patients with sickle cell trait even though the incidence is low as compared with sickle cell anaemia [6–9]. However, so far priapism in Hb E homozygosity is not reported in literature. In this correspondence we intend to bring to notice an unusual association of priapism with Hb E homozygosity. A 27 years old male, resident of North Eastern part of India was referred to our haematology OPD to rule out any underlying haematological disorder as the individual had an episode of priapism 3 months ago. The patient had an episode of unprovoked painful persistent erection for more than 8 h for which he sought medical advice. The surgeon at that medical center had managed with therapeutic needle aspiration from corpora cavernosa combined with flushing of cavernosa with normal saline to clear the sludged blood. The patient’s symptom subsided subsequently with the intervention and was referred to our center for further evaluation. On detailed clinical history, the individual was not on any medication for chronic illnesses; neither was he abusing any psychoactive drugs, alcohol, no history of prior trauma to the perineal region. His physical examination revealed mild pallor. There was no hepatosplenomegaly or lymphadenopathy. Systemic examination did not reveal any abnormality. On USG abdomen, spleen was not reported as enlarged (Span 13 cms). On investigations, haemoglobin, total leucocyte count and platelet count were 9.1 gm/dL, 7.1 9 10/lL and 118 9 10/lL respectively. Peripheral smear examination revealed microcytic hypochromic red cells and target cells with normal differential leucocyte count and reticulocyte count of one percent. There were no sickle cells or atypical cells in peripheral smear and sickling test was also negative. Subsequently Hb HPLC was performed which revealed with Hb A2 ? E of 92.6 % (Retention time3.68 min), Hb A of 6 % (Retention time 2.29 min) and Hb F (Retention time 1.06 min) of 1.6 % (Fig. 1; Table 1). This was followed by parental study which revealed Hb A2 ? E in mother and father of 29 and 27 % respectively suggestive of both parents being heterozygous for Hb E, S. Venkatesan A. Purohit (&) M. Aggarwal P. K. Singh T. Seth H. P. Pati Department of Hematology, All India Institute of Medical Sciences, New Delhi, India e-mail: purohitabhi80@gmail.com

A young adult diagnosed as T-acute lymphoblastic leukemia presented with unusual complications during chemotherapy. He was diagnosed to have tubercular splenic abscess and had neurologic complaints like vincristine- induced peripheral neuropathy and focal neurologic deficit complicating the course of chemotherapy. Cases of hepatosplenic tuberculosis presenting as abscesses during prolonged neutropenia in patients with acute leukemia during or after chemotherapy are rare but reported. Vincristine induced neuropathy is reported to be higher in Indian children possibly due to co-existent malnutrition. The focal lesions in the brain were intriguing as regards to the symptomatology and exact etiology especially with the limitation of imaging in such patients. This case highlights the difficulty in the course of chemotherapy due to the rare complications encountered.

Pleural effusions in acute myeloid leukemia (AML) can have wide differential diagnosis, however AML presenting as pleural effusion with leukemic infiltration is rarely documented. A 22 year old male presented with pleural effusion for 3 months and subsequently diagnosed as AML M2, which prompted us for this communication.

Diagnosis of immune thrombocytopenia (ITP) is based on clinical suspicion and normal peripheral smear except for thrombocytopenia. Bone marrow examination is carried out to rule out leukemia, myelodysplastic syndrome or aplastic anemia. However, in most cases, clinical diagnosis is not altered after the bone marrow reports. Hence, this present study was carried out to evaluate the justification for bone marrow examination in the setting of isolated thrombocytopenia. All patients presenting to the hematology OPD with isolated thrombocytopenia and suspected diagnosis of ITP, between October 2011 and April 2013, were included in the study. Data was collected from bone marrow reports and outpatient records. A total of 353 cases were found. 319 cases had features of typical ITP and the rest had some form of organomegaly and/or lymphadenopathy. Bone marrow examination in all cases revealed normal hematopoietic elements and prominence of megakaryocytes including juvenile forms with no novel diagnosis in any patient. Routine use of bone marrow examination in the diagnostic workup of isolated thrombocytopenia is not required in our center even if steroids are planned as a first line therapy. However, a detailed history, thorough examination with complete hemogram and peripheral smear examination are essential.

Histoplasmosis is a systemic mycosis which has a worldwide distribution.Though common in immunocompromised patients, disseminated histoplasmosis has rarely been reported in patients with aplastic anemia. We report here a case of very severe aplastic anemia who developed disseminated histoplasmosis in the form of hepatic involvement, which proved fatal due to lack of cell mediated immunity.

Dear Editor, Infarcts are commonly seen in spleen, and rarely in myocardium and brain, in patients of chronic myeloid leukemia (CML). Bone infarcts, manifesting as severe debilitating pain as an initial presentation of CML have not been reported. We present a case of CML in chronic phase whose initial presentation was severe bone pains with inability to walk. Tc-99m MDP bone scan revealed heterogenous areas of bone infarct involving long bones of both upper and lower limbs. She was treated with Imatinib and Ecosprin. She became complete pain-free in 4 months. This case highlights the unusual presentation of CML-CP and complete recovery with standard therapy. A 32-years old woman presented with diffuse bony-pains involving bilateral upper and lower limbs for 3 weeks duration. She was not able to perform her daily routine activities because of severe pain (ECOG performance status 3). Clinical examination revealed mild pallor and splenomegaly (spleen size 8 cm below costal margin). Hemogram revealed hemoglobin 9.4 g/dl, total leukocyte count 136 × 109/l, platelet count 588 × 109/l and differential count-neutrophils 16%, lymphocytes 22%, myelocytes 24%, metamyelocytes 34%, basophils 3% and blasts 1% (Sokal, Intermediate risk 0.83). Her biochemical profile revealed blood urea 36 mg/dl, creatinine 1.1 mg/dl, uric acid 4.2 mg/dl, calcium 8.6 mg/dl and bilirubin 0.8 mg/dl. Her leukocyte alkaline phosphatase (LAP) score was low and RT-PCR for BCR-ABL was positive for p210. She was diagnosed as a case of chronic myeloid leukemia- Chronic phase (CML-CP). Radiographs of limb bones were normal. Tc-99m MDP bone scan revealed heterogenous increased radiotracer uptake in limb bones (involving bilateral humeri, forearm bones, femurs), suggestive of multiple bone infarcts. Rest of the skeleton was normal. She was started on hydroxyurea 500 mg thrice daily, allopurinol 100 mg thrice daily, imatinib mesylate 400 mg once daily and ecosprin 75 mg once daily. She was given analgesics (paracetamol and tramadol) for bone pains. Hydroxyurea and allopurinol were stopped after 2 weeks and imatinib and ecosprin continued. Patient achieved complete hematological response after 2 months of imatinib therapy but continued to have limb pains for 4 months though with decreased intensity. Pain completely subsided 5 months later (ECOG performance status 0). Repeat bone scan performed at 5 months revealed completely normal bone scan. Quantitative PCR for BCR-ABL at 15 months revealed major molecular response. CML accounts for approximately 15 to 20% of leukemias in adults. The clinical findings at diagnosis of CML-CP vary among reported series. Twenty to 50% of patients are asymptomatic, with the disease first being suspected from routine blood tests [1, 2]. Among symptomatic patients, systemic symptoms such as fatigue (34%), weight loss (20%), abdominal fullness (15%) and bleeding episodes due to platelet dysfunction (21%) are common [2]. Abdominal pain and discomfort may include left upper quadrant pain and early satiety due to the enlarged spleen with or without perisplenitis and/or splenic infarction. Tenderness over the lower sternum, due to an expanding bone marrow, is sometimes seen. Acute gouty arthritis may also present due to overproduction of uric acid [3]. Other frequent findings include splenomegaly (present in 48–76%), anemia (45–62%), white blood cell count above 100 × 109/l (52–72%) and platelet count above 600 × 109 (15–34%) [1, 2]. Involvement of extramedullary tissues such as the lymph nodes, skin, and soft tissues is generally limited to patients with blast crisis. Although thrombocytosis is common, thrombosis is not seen [2]. Other rare manifestations include splenic rupture [4] and priapism [5]. The radiological changes studied in CML include diffuse osteoporosis, focal osteolytic and osteoblastic lesions, chloromas and arthritis [6]. Bone pains are one of the common presentations of hematological malignancies [7] and are usually due to marrow infiltration by leukemic cells causing expansion of bone marrow. Bone infarction with severe pain as initial presentation has never been reported in CML-CP, though a case of CML developing bone infarction and progressing to myelofiberosis has been documented [8]. Our patient presented with diffuse debilitating bone pains due to multiple bone infarcts which is an unusual presentation of CML-CP. The patient responded to the usual treatment with complete disappearance of bone lesions and complete clinical recovery.