Pancytopenia with aplastic anemia in systemic lupus erythematosus: case series and literature review.

Aplastic anemia (AA) is a rare, potentially catastrophic hematopoiesis failure manifested by pancytopenia and bone marrow aplasia. AA occurrence in Systemic Lupus Erythematosus (SLE) patients is extremely rare. The diagnosis may be delayed due to other possible pancytopenia etiologies. Confirmation of peripheral cytopenias diagnosis necessitates a bone marrow aspiration. The management of AA is challenging, and the literature reported using glucocorticoids, danazol, plasmapheresis, cyclophosphamide, intravenous immunoglobulin, and cyclosporine. We report two cases of SLE patients who presented with pancytopenia, with bone marrow biopsy confirmed AA. One case was treated with cyclophosphamide but unfortunately succumbed to Acute Respiratory Distress Syndrome (ARDS), while the other case was managed with rituximab with a good response. Interestingly, both patients were on azathioprine before the diagnosis of AA. A comprehensive search for reported cases of AA in PubMed, Scopus, and the Directory of Open Access Journals databases was performed to enhance the understanding of the diagnostic and management challenges associated with AA in SLE, facilitating ongoing exploration and research in this field. The decision to do a BM aspiration and biopsy is recommended for SLE patients with an abrupt decline in blood counts and previously stable blood counts.

Aplastic anaemia following antibiotic use for urinary tract infection.

Aplastic anaemia is often associated with recent viral illnesses to include EBV and parvovirus along with certain medications such as anticonvulsants and sulfa containing antibiotics. We describe a case report of a female patient in her 70s who presented with pancytopenia after being treated with nitrofurantoin and ciprofloxacin for suspected urinary tract infection. She underwent an extensive workup to rule out alternative aetiologies of her pancytopenia to include a broad viral, autoimmune and malignancy evaluation which were unrevealing. Given her recent exposure to ciprofloxacin and nitrofurantoin and marrow recovery following removal of these agents, it was presumed that antibiotic exposure was the underlying cause of her aplastic anaemia.

[Hemophagocytic lymphohistiocytosis: A retrospective analysis of 66 patients]. / Lymphohistiocytose hémophagocytaire : analyse rétrospective de 66 patients.

CONTEXT: Hemophagocytic lymphohistiocytosis is a rare syndrome with a poor prognosis, characterized by an uncontrolled dysregulation of the immune system. The rarity of this disease makes it difficult to obtain large cohorts. In this study, we analyzed the data of 66 patients: the objective was to describe the epidemiological, clinical, biological and therapeutic characteristics and to compare our results with those already published. METHODS: We conducted a retrospective study at the University Hospital of Montpellier from 2015 to 2021. Patients were included when the diagnosis of HLH was mentioned on the hospitalization report and when the HSCORE was higher than 50% (169). Prognostic analyses were performed by comparing the patients who died from HMH to those who didn't. RESULTS: The mean age the 66 patients included was 49.2 years, 62% were men. The percentage of deaths was 45.9%. Lymphoma was the main etiology, followed by infections, then autoimmune/autoinflammatory diseases. Fever, splenomegaly, hepatomegaly and organ failure were the main clinical manifestations. Pancytopenia was present in 62% of cases. Ferritin, triglycerides, LDH and AST were highly increased. Advanced age, associated lymphoma, and the severity of cytopenias were linked to a poor prognosis. DISCUSSION: The study of the clinico-biological, epidemiological and survival data of the patients in our cohort allowed us to confirm previously published data but also to discuss some of them.

Undiagnosed tuberculosis associated with hemophagocytic lymphohistiocytosis due to improper use of corticosteroid.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening hematologic disease segregated into familial (primary) and acquired (secondary) subtypes. Hyperinflammation and HLH occur when the immune system fails to clear activated macrophages and histiocytes. Infections, malignancies, and rheumatologic disorders are the major triggers leading to HLH. Miliary tuberculosis is a serious disease with a lymphohematogenous spread of Mycobacterium tuberculosis, which is known to be one of the causative agents of HLH. Miliary tuberculosis and HLH have atypical presentations which are similar to routine diseases. Hence, physicians may face challenges to diagnose and treat these complications. CASE REPORT: We report the case of a 60-year-old man with a history of prolonged fever, shortness of breath, jaundice, altered mental status, undiagnosed lower back pain, and overuse of parenteral betamethasone. Miliary tuberculosis was diagnosed by diffuse, vague random micronodules in both lungs and positive acid-fast bacilli in bronchoalveolar lavage and bone marrow aspiration and biopsy. Moreover, compatible presentation and pancytopenia, hypertriglyceridemia, high serum level of ferritin and fibrinogen-derived products, and evidence of hemophagocytosis on bone marrow aspirate led to the diagnosis of HLH. Unfortunately, despite nearly two months of an anti-tuberculosis regimen (standard and salvage) and eight doses of etoposide, he eventually passed away after clinical improvement. CONCLUSIONS: Irrational and indiscriminate use of glucocorticoids can be a devastating cause of the spread of tuberculosis and its rare complications, such as HLH.

A New Case of Association of Megaloblastic Anemia and Pancytopenia of Infants.

BACKGROUND: Vitamin B12, or cobalamin deficiency, an infrequent clinical entity in pediatric age, is found almost solely in breastfed infants whose mothers are purely vegetarian, non-supplemented or with pernicious anemia. Megaloblastic anemia in infants presents with generalized weakness or irritability. METHODS: Diagnosis is usually centered on complete blood count, vitamin dosing, and peripheral smear, which may show macrocytes, hypersegmented neutrophils, reticulocytopenia and a raised mean corpuscular volume (MCV ˃ 100 fL). Pancytopenia has also been noted. RESULTS: We report an exclusive breastfed nine-month-old female child who presented with irritability, developmental delay, and difficulties in introducing new foods. Her initial blood count revealed pancytopenia. Vitamin B12 levels were found to be reduced. Maternal levels of Vitamin B12 were also found to be borderline low. The child was treated as per protocols, and improvement was evidenced with the return of hematological parameters to the regular and gradual advancement of milestones. CONCLUSIONS: We aim to underscore the importance of megaloblastic anemia as an important and rare cause of anemia in infancy.

Impact of secondary infections on dengue presentation: A cross-sectional study in a tertiary care hospital in Uttar Pradesh, India.

BACKGROUND: Recently, a wide range variety of manifestations, including a self­limiting to severe illness, has been increasingly reported in dengue. Few studies attract attention to severe dengue, mainly observed in secondary infection. With this background, this study aims to provide a comprehensive overview to differentiate primary from secondary dengue using serology (IgG) and the possible association of severity of illness in secondary dengue. METHODS: Present retrospective cross-sectional study was conducted at a North Indian tertiary care center from September 2021 to January 2022. Clinical data of confirmed dengue patients from the medicine department were collected and assigned as primary and secondary dengue. RESULTS: Of the 220 dengue patients, 22 (10 %) had secondary dengue infection. Hemorrhagic manifestations were reported in 58/220 (26.4 %) cases while 7/22 (31.8 %) in secondary dengue. Prevalent hemorrhagic manifestations in secondary dengue include purpura (27.3 %), vaginal bleeding (4.5 %), melaena (9.1 %), and epistaxis (4.5 %). In addition, 42 (19.1 %) patients had pancytopenia, and 8 (36.6 %) cases were of secondary dengue. Hepatic dysfunction was noted in 164 (74.5 %) cases. Notably, all secondary dengue cases (22;100 %) had hepatic dysfunction and severe in 9 (40.9 %) cases. In addition, in secondary dengue patients, evidence of plasma leakages such as hypoproteinemia 7 (31.8 %) and ascites (35 %) were statistically more frequent. Overall, two deaths (0.9 %) were reported, and were one in each group. CONCLUSIONS: Many parameters, including hemorrhagic manifestation (melaena), hematological characteristic (pancytopenia), evidence of plasma leakage (hypoproteinemia and ascites), gastrointestinal (GB wall thickening and hepatic dysfunction) and reduction in mean hemoglobin and platelet count were found to be statistically significant in secondary dengue infection. Additionally, early classification of secondary dengue may help to anticipate its severity and allow for early strategic intervention/management to lower morbidity and mortality.

[Pancytopenia associated to acute myeloid leukemia: a case report of aleukemic leukemia]. / Pancitopenia asociada a leucemia mieloide aguda: reporte de un caso de leucemia aleucémica.

Background: Acute myeloid leukemia (AML) is characterized by the presence of ≥ 20% myeloblasts in peripheral blood or bone marrow, as well as specific cytogenetic alterations. It can appear as a de novo disease or be associated with other hematologic diseases, which is why the clinical presentation is heterogeneous. Pancytopenia as a manifestation of aleukemic leukemia is a rare entity. Here, we described a case of AML that presented with pancytopenia as the only manifestation in a secondary care center. Clinical case: 72-year-old man, hospitalized due to pancytopenia, with no history of hematological diseases, asymptomatic, without hepatosplenomegaly or bleeding. Flow cytometry revealed pancytopenia without blasts in peripheral blood. Secondary causes of pancytopenia as infections, splenomegaly and nutritional deficiencies where ruled out. Bone marrow aspirate showed infiltration by 45% of myeloblasts and myelodysplasia. Immunophenotype was compatible with AML. Patient was sent to the Hematology Department at Centro Médico Nacional Siglo XXI (21st Century National Medical Center) to start chemotherapy. Conclusions: AML that is presented as pancytopenia should be considered in the evaluation of marrow failure syndrome. In the context of our hospital, morphological findings remains an essential tool for early diagnosis, since more refined studies such as immunophenotyping and cytogenetic testing are unreachable in a timely manner.

Introducción: La leucemia mieloide aguda (LMA) se caracteriza por presentar ≥ 20% de mieloblastos en sangre periférica o médula ósea, así como alteraciones citogenéticas específicas. Surge como enfermedad de novo o asociada a trastornos hematológicos, por lo que la presentación clínica es heterogénea. La presentación como pancitopenia (leucemia aleucémica) es rara. El objetivo de este trabajo es presentar un caso de LMA que cursó con pancitopenia como única manifestación clínica en un hospital de segundo nivel de atención. Caso clínico: hombre de 72 años, hospitalizado por hallazgo de pancitopenia, sin historial de enfermedades hematológicas, asintomático, sin adenomegalias ni hemorragia. La citometría hemática documentó pancitopenia sin blastos en sangre periférica. Se descartaron causas secundarias como infección, esplenomegalia y deficiencias nutricionales. En el aspirado de médula ósea se observó 45% de mieloblastos y mielodisplasia. El inmunofenotipo fue compatible con LMA. El paciente fue referido a Hematología del Centro Médico Nacional Siglo XXI para iniciar quimioterapia. Conclusiones: la LMA que se presenta como pancitopenia debe ser tomada en cuenta en el protocolo diagnóstico de síndrome de falla medular. En el contexto de nuestro hospital, la morfología hematológica sigue siendo una herramienta indispensable para el diagnóstico temprano de este tipo de enfermedades, ya que estudios más sofisticados, como el inmunofenotipo y la citogenética, no se encuentran disponibles de forma oportuna.

Research progress on the hematopoietic microenvironment in aplastic anemia.

Aplastic anemia (AA) is a disease of bone marrow hematopoietic failure, and the main clinical manifestation is pancytopenia. Its pathogenesis is still unclear. In recent years, more research has been done on its immune abnormalities to explain its pathogenesis and less on the hematopoietic microenvironment, but there are still some advances. This article summarizes the research on the hematopoietic microenvironment of AA in recent years to provide new ideas for the clinical treatment of AA.

Vitamin B12 deficiency presenting with a pancytopenia in pregnancy.

Vitamin B12 deficiency is a significant public health problem globally. Although it is a well-known cause of macrocytic anaemia and in advanced cases, pancytopenia, there remains a relative paucity of cases reported in pregnancy. It is associated with an increased risk of pregnancy complications and adverse birth outcomes such as neural tube defects, preterm birth, low birth weight, neurological sequelae and intrauterine death. It has a predilection for individuals aged >60 years. It has been implicated in a spectrum of neuropsychiatric disorders and it may also exert indirect cardiovascular effects. Severe vitamin B12 deficiency may present with haematological abnormalities that mimic thrombotic microangiopathy such as HELLP syndrome (haemolysis, elevated liver enzymes and low platelets) or it may present as pseudothrombotic microangiopathy (Moschcowitz syndrome) characterised by anaemia, thrombocytopenia and schistocytosis. It can also closely mimic thrombotic thrombocytopenia purpura, hence posing a diagnostic challenge to the unwary physician. Serological measurement of vitamin B12 levels confirms the diagnosis. Oral supplementation with vitamin B12 remains a safe and effective treatment. The authors describe the case of a multiparous woman in her late 20s presenting with a plethora of non-specific symptoms at 29+5 weeks' gestation. Her haemoglobin was 45 g/L, platelets 32×109/L, vitamin B12 <150 ng/L and serum folate <2 µg/L. She was not a vegetarian, but her diet lacked nutrition. Following parenteral B12 supplementation, her haematological parameters improved. The pregnancy was carried to term. Due to the plethora of non-specific symptoms, the diagnosis can be challenging to establish. Adverse maternal or fetal outcomes may occur. Folic acid supplementation may mask an occult vitamin B12 deficiency and further exacerbate or initiate neurological disease.

Use of Colony-Stimulating Factors in Patients With Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that commonly manifests as cutaneous rashes, renal disease, gastrointestinal dysfunction, and cytopenia. Hematological anomalies are frequently associated with drug-induced toxicity in SLE patients. Colony-stimulating factors have been used to treat drug-induced cytopenia in past case reports; however, evidence suggests that colony-stimulating factors can exacerbate autoimmune disorders, including SLE. This case report presents two patients with SLE exacerbations after colony-stimulating factor administration. The first case is a young male with SLE who developed pancytopenia with a white blood cell count (WBC) of 1 × 109 cells/L. The patient was administered filgrastim during his initial admission and presented to the hospital 2 days after discharge in cardiac arrest with a WBC of 66.7 × 109 cells/L. The second case is a 49-year-old female with SLE who was administered sargramostim in response to a WBC count of 9 × 109 cells/L. The patient experienced a drastic increase in WBC followed by a cardiac arrest. These cases highlight the need for more research regarding the safe use of colony-stimulating factors in SLE patients.

Gastro-colic Fistula-associated Hypersplenism Causes Pancytopenia in a Patient with Crohn's Disease.

A 24-year-old woman was admitted to our hospital due to abdominal pain and a high fever. She was diagnosed with ileocolonic Crohn's disease (CD), complicated with a gastro-colic fistula and splenomegaly. After initial treatment with an infliximab-biosimilar, all blood cell line counts markedly decreased. Three-dimensional reconstructed computed tomography revealed splenic vein narrowing. Thus, her pancytopenia was deemed to have likely been caused by hypersplenism. Surgery was performed, and clinical remission was maintained without pancytopenia. This is the first report of a CD patient with pancytopenia caused by hypersplenism that was triggered by gastro-colic fistula-associated splenic vein obstruction.

Disseminated Tuberculosis in an Immunocompetent State: A Case Report.

BACKGROUND: Tuberculosis is one of the major infectious diseases of mankind and remains a significant health concern, especially in developing countries. Clinical manifestations of TB are broad and sometimes very challenging for clinicians to diagnose early. Tuberculous psoas abscess was generally secondary to spinal tuberculosis or direct extension from adjacent structures in immunocompromised individuals, but tuberculous psoas abscess in the immunocompetent state is very infrequent. In addition, pancytopenia and new onset neck swelling simultaneously make this presentation a very unusual clinical entity in tuberculosis. CASE PRESENTATION: We now present a case of a 21-years-old, unmarried, otherwise healthy girl presented with fever, lower abdominal pain and weight loss for two months. She also noticed painless neck swelling for 15 days. She later had a tuberculous left sided psoas abscess with pancytopenia and a cold abscess on the left side of the neck with no sign of any other apparent focus, according to the evidence. Diagnosis of disseminated TB without lung involvement was established and ATT was started. The outcome was successful on follow up. CONCLUSION: Among the broad spectrum of atypical manifestations of TB, this case report draws attention to its rarity, diagnostic challenge and awareness of the clinical spectrum, especially in developing countries.

How I diagnose and treat acute graft-versus-host disease after solid organ transplantation.

Acute graft-versus-host disease (GVHD) is a rare complication after solid organ transplantation (SOT) that carries high mortality. Caused by immunocompetent donor leukocytes within the transplanted organ, which become activated against recipient tissues, GVHD typically develops 2 to 12 weeks after SOT and can affect the skin, gastrointestinal tract, liver, and bone marrow. Signs and symptoms are nonspecific and include a rash, nausea, appetite loss, diarrhea, and cytopenias. Pancytopenia from marrow-directed GVHD is the primary driver of mortality. The diagnosis of GVHD is often delayed but should be confirmed by biopsy of an affected organ. Evidence of donor chimerism in blood or marrow supports the diagnosis. When GVHD is diagnosed we initiate treatment with systemic corticosteroids. At that time, if GVHD only involves skin or oral mucosa we also decrease maintenance immunosuppression levels to allow the recipient to reject the donor immune cells. For GVHD involving the marrow we initiate an allogeneic hematopoietic cell donor search early. In this article, we describe 3 cases of GVHD after SOT, outline our approach to diagnosis and management, and then provide analysis of the 3 instructive cases.

Bone marrow oxalosis with pancytopenia in a patient with short bowel syndrome: Report of a case and review of the literature.

Systemic oxalosis is a condition in which calcium oxalate crystals deposit into various bodily tissues. Although this may occur as the result of a rare primary syndrome in which an error of glyoxylate metabolism causes an overproduction of oxalate, it is more often seen as a secondary process characterized by increased enteric oxalate absorption. Here, we describe a patient with short bowel syndrome on long-term parenteral nutrition support who developed a unique manifestation of systemic oxalosis, leading to deposition of oxalate crystals within the bone marrow contributing to pancytopenia. In this report, in addition to reviewing the literature on this presumably rare manifestation of oxalosis, we also discuss its pathogenesis in the setting of short bowel syndrome and its management, including prevention.

Leukemic retinopathy presenting as concurrent bilateral subhyaloid hemorrhage and subarachnoid hemorrhage in a patient with acute monocytic leukemia: a case report.

BACKGROUND: Ophthalmic manifestations are common in patients with leukemia, developing in nearly 50% of cases. Intracranial hemorrhage is another potentially fatal complication of leukemia. In this case report, we aim to present a challenging case that involves both ophthalmic and intracranial manifestations in an individual with acute monocytic leukemia. CASE PRESENTATION: A 36-year-old Persian male presented to the emergency room with complaints of fever, headache, and bilateral blurred vision. The patient had been diagnosed with acute monocytic leukemia 3 months prior and had undergone four sessions of induction chemotherapy, the last of which was 10 days prior to admission. The patient was admitted to the internal medicine service, and initial lab studies confirmed pancytopenia, including severe neutropenia, anemia, and thrombocytopenia. Subarachnoid hemorrhage in the left frontal lobe was detected through spiral brain computed tomography scan. Ophthalmic examination revealed visual acuity of light perception in the right eye and 3-m finger count in the left eye. Fundus examination revealed bilateral peripapillary subhyaloid and intraretinal hemorrhages, confirming leukemic retinopathy. The patient showed significant improvement in visual acuity and hemorrhage resolution through conservative treatment and regular follow-ups after 3 months. CONCLUSION: Simultaneous subarachnoid hemorrhage and bilateral subhyaloid hemorrhages seemed to have occurred as a result of pancytopenia. Management approach of ophthalmic manifestations of leukemia involves interdisciplinary cooperation and should be individualized on the basis of the patients' underlying medical condition.

Severe alveolar bone resorption in Felty syndrome: a case report.

BACKGROUND: Felty syndrome is defined by three conditions: neutropenia, rheumatoid arthritis, and splenomegaly. Neutropenia associated with pancytopenia may further affect the dental condition of a patient. Periodontal treatment and surgery in patients with Felty syndrome necessitates cooperation with a hematologist. Here we present a case of a patient with Felty syndrome who was initially referred to the oral surgery hospital attached to the School of Dentistry for extensive periodontitis. She was effectively treated in collaboration with the hematology department. CASE PRESENTATION: A 55-year-old Asian woman visited our department with concerns of worsening tooth mobility, discomfort, and spontaneous gingival bleeding. Initial periodontal examination revealed generalized severe periodontitis (Stage IV Grade C) resulting from leukopenia/neutropenia and poor oral hygiene. A thorough treatment strategy involving comprehensive dental procedures, such as multiple extractions and extensive prosthetic treatment, was implemented. Following the diagnosis of Felty syndrome, the patient was started on treatment with oral prednisolone 40 mg/day, which effectively controlled the disease. Furthermore, there was no recurrence of severe periodontitis after the periodontal treatment. CONCLUSIONS: Dentists and physicians should be aware that immunocompromised individuals with pancytopenia and poor oral hygiene are at risk of developing extensive periodontitis. If their susceptibility to infection and pancytopenia-related bleeding can be managed, such patients can still receive comprehensive dental treatment, including teeth extractions and periodontal therapy. Cooperation among the dentist, hematologist, and patient is necessary to improve treatment outcomes and the patient's quality of life.

Complicated appendicitis in a patient with myelodysplastic syndrome and pancytopenia.

ABSTRACT: Patients with immunocompromise presenting with abdominal pain can present a diagnostic challenge, because they may have atypical or vague symptoms. This case report discusses nonoperative management of a patient with immunocompromise, complicated appendicitis, and intra-abdominal infection.

Graves' hyperthyroidism induced pancytopenia, epilepsia and muscle weakness: A case report.

RATIONALE: Pancytopenia and epilepsia are rare complications of Graves' disease (GD). Muscle weakness is a physical sign of GD. It is extremely rare for GD patients to present 3 symptoms at the same time. PATIENT CONCERNS: A 35-year-old female was admitted to hospital for dizziness for 1 day. The results of laboratory examination on admission showed pancytopenia and hypothyroidism. Her clinical manifestations include pancytopenia, epilepsy, and muscle weakness. DIAGNOSIS: Graves' hyperthyroidism. INTERVENTIONS: She received endotracheal intubation, ventilator, antithyroid drugs, and hormone therapy. OUTCOME: The patient was discharged after treatment. LESSON: Severe complications caused by GD are rare and require antithyroid therapy. Although glucocorticoid is not recommended by the guidelines, it can effectively improve thrombocytopenia.

When refeeding is not enough: severe and prolonged pancytopenia in an adolescent with anorexia nervosa.

A 15-year-old female patient with anorexia nervosa presented an unusually prolonged and severe episode of pancytopenia with severe thrombopenia and severe leucopenia. Despite effective refeeding, active specialized interventions were necessary. Upon admission, the patient presented with severe and symptomatic thrombopenia, severe neutropenia and gelatinous marrow transformation. In addition to refeeding, active interventions such as platelet transfusion and granulocyte-colony stimulating factor were successful to manage the patient's complications. The etiological search for pancytopenia was negative. The patient's prolonged starvation was probably a key factor. Medical history, clinical presentation, evolution, and biological data including bone marrow aspiration results are presented. Management of cytopenia and of their complications in a context of severe starvation is discussed in regard of existing literature. A simple monitoring attitude may prove insufficient in cases of severe pancytopenia in anorexia nervosa.Level of evidence V, descriptive study.