Comparison of preoperative anxiety, bruxism, and postoperative pain among patients undergoing surgery for septoplasty, endoscopic sinus surgery, and tympanoplasty.

Background: Septoplasty, endoscopic sinus surgery, and tympanoplasty are the most commonly performed elective functional ear-nose-throat surgeries. Aim: This study investigated the relationship between preoperative anxiety, bruxism, and postoperative pain in inpatient groups undergoing three different functional otorhinolaryngologic surgeries. Patients and. Methods: This study was conducted in a single center of a tertiary referral hospital. The patients (n = 90) who had undergone septoplasty (group A), endoscopic sinus surgery (group B), and tympanoplasty (group C) were included. The State-Trait Anxiety Inventory (STAI) questionnaire and the Amsterdam Preoperative Anxiety Information Scale (APAIS) were administered. To evaluate bruxism, a self-questionnaire was administered, and for the evaluation of pain, the visual analogue scale (VAS) was administered. Results: In group C, preoperative STAI and APAIS and early and late pain values were higher than in the other groups. When patients were divided into two groups according to the presence of bruxism. A significant difference was found between the preoperative STAI and immediate and late VAS values (P < 0.001). A strong correlation was observed between APAIS and early and late VAS values in group C (P < 0.001). Conclusion: Patients who will undergo tympanoplasty should be aware of the preoperative anxiety level and pain follow-up. Bruxism can be considered a vital follow-up parameter that manifests due to high preoperative anxiety. It may also be useful to examine preoperative bruxism and take appropriate measures due to its pain-increasing effect in patients.

Cutaneous drug reactions to antiepileptic drugs and relation with HLA alleles in the Turkish population.

Summary: Background and objective. Many studies have shown associations between HLAB*15:02, HLA-A*31:01 and carbamazepine (CBZ)-induced delayed cutaneous hypersensitivity reactions. The aim of this study is to evaluate a possible association between delayed cutaneous reactions to antiepileptic drugs (AEDs) and certain HLA-A and HLA-B alleles in the Turkish population. Methods. The study consisted of 3 groups: Group I (reactive group) included the patients who had documented delayed cutaneous reactions to any antiepileptic drug. Group II (non-reactive group) included the patients who have been on antiepileptic treatment at least for three months without any adverse reactions. Group III consisted of healthy subjects. The HLA-A and B alleles were analyzed in all groups. Results. Forty patients (29 female) had experienced different hypersensitivity reactions due to AEDs: maculopapular exanthema (26 patients), Stevens-Johnson syndrome (6 patients), drug rash with eosinophilia and systemic symptoms (7 patients), toxic epidermal necrolysis (1 patient). Lamotrigine (11) and CBZ (10) were the most common culprit drugs involved in the reactions. The HLA-B*15:02 was not present in any of the study groups. However, HLA-B*35:02 was found in 4 patients from the reactive group, while it was not observed in non-reactive patients and was detected in only one healthy subject (p = 0.021). Conclusion. Although our preliminary results did not indicate a strong allele association with AED hypersensitivity, HLA-B*35:02 appears to be a candidate allele for MPE / DRESS / DIHSS induced by AED's in Turkish population. Further studies with a larger sample size may result in more comprehensive data about the genetic tendency for AED hypersensitivity in the Turkish population.

How do voice restoration methods affect the psychological status of patients after total laryngectomy?

OBJECTIVE: This study investigated the relationship between psychological well-being and different voice rehabilitation methods in total laryngectomy patients. METHODS: The study enrolled 96 patients who underwent total laryngectomy. The patients were divided into three groups according to the voice rehabilitation method used: esophageal speech (24 patients); a tracheoesophageal fistula and Provox 2 voice prosthesis (57 patients); or an electrolarynx (15 patients). The participants were asked to complete the Turkish version of the Voice Handicap Index-10 (VHI-10) to assess voice problems. They were also asked to complete the Turkish version of the Perceived Stress Scale (PSS), and the Hospital Anxiety and Depression Scale (HADS). The test scores of the three groups were compared statistically. RESULTS: Patients who used esophageal speech had a mean VHI-10 score of 10.25 ± 3.22 versus 19.42 ± 5.56 and 17.60 ± 1.92 for the tracheoesophageal fistula and Provox 2 and electrolarynx groups respectively, reflecting better perception of their voice. They also had a PSS score of 11.38 ± 3.92, indicating that they felt less stressed in comparison with the tracheoesophageal fistula and Provox 2 and electrolarynx groups, which scored 18.84 ± 5.50 and 16.20 ± 3.49 respectively. The HADS scores of the groups were not different, indicating that the patients' anxiety and depression status did not vary. CONCLUSION: Patients who used esophageal speech perceived less stress and were less handicapped by their voice.

The clinicopathological features and survival of Castleman disease: a multicenter Turkish study.

OBJECTIVE: In this study, we aimed to investigate the clinicopathological features and survival of CD, which is quite rare and has many unknowns. PATIENTS AND METHODS: This study was conducted by retrospectively evaluating patients diagnosed with CD in six different centers in Turkey. RESULTS: The median age of 33 patients included in the study was 49 and 51.5% (n = 17) of these patients were women. 18 (54.5%) patients were in the hyaline vascular subtype and most of the patients were UCD (n = 20, 60.6%). The most common involvement region was head and neck (n = 19, 57.5%). The UCD group was younger than the MCD group (p=0.027). Visceral lymph node involvement was higher in MCD than in UCD (p=0.001). Similarly, it was observed that there was more hepatomegaly (p=0.035) and splenomegaly (p=0.013) in the MCD group. During the median 19.5 months follow-up period, there were no patients who died. CONCLUSIONS: It was observed that UCD and MCD are different clinical entities. Promising survival times can be achieved with surgical and systemic treatments in both subtypes of this extremely rare disease. However, this result should be supported by well-designed prospective comprehensive studies.

Nasal eosinophilia in nasal smears of patients with persistent and intermittent allergic rhinitis.

BACKGROUND: Eosinophils are the principal effector cells involved in the pathogenesis of allergic rhinitis. Cell numbers increase in non-allergic rhinitis patients with eosinophilia, aspirin hypersensitivity and nasal polyposis, as well as in allergic rhinitis patients. Exfoliative nasal cytology can be used in the differential diagnosis of allergic rhinitis. OBJECTIVE: To evaluate nasal eosinophilia in nasal smears of patients with mild, persistent and intermittent allergic rhinitis. METHODS: The study comprised 60 patients with allergic rhinitis and 20 healthy volunteers. The patients were divided into intermittent and persistent allergic rhinitis groups. Nasal smear status, eosinophil numbers and Total Nasal Symptom Scores were compared. RESULTS: Nasal smear results were pathological in 40 of 60 allergic rhinitis patients, which was significantly higher than the rate in controls. The mean nasal eosinophilia score was significantly higher in the intermittent allergic rhinitis than in the persistent allergic rhinitis group (p = 0.029). There was a positive correlation between nasal eosinophilia score and Total Nasal Symptom Score (r = 0.652; p < 0.05) in persistent allergic rhinitis and intermittent allergic rhinitis patients. CONCLUSION: The nasal smear test is inexpensive, objective and simple to perform, and should be part of the diagnostic investigation.

Facial motor cortex plasticity in patients with unilateral peripheral facial paralysis.

Hand motor representation area expands towards the area of the perioral facial motor cortex in patients with peripheral facial paralysis (PFP) and in hemifacial spasm cases treated with botulinum toxin. In this current study, we aimed to investigate the changes both in the ipsilateral and contralateral facial motor cortex areas in patients with PFP with transcranial magnetic stimulation (TMS). Thirty healthy individuals and 41 patients with unilateral PFP with partial or total axonal degeneration participated in this study. Motor evoked potentials (MEPs) of perioral muscles elicited by TMS of the intracranial portion of the facial nerve and motor cortex, were recorded. TMS was delivered through a figure-of-eight coil. Mapping of the cortical representation of perioral muscles were also studied in 13 of 41 patients and in 10 of control subjects. Mean amplitude of the intact perioral MEPs elicited by the ipsilateral hemisphere TMS, was significantly higher in patients than the control subjects. There was also a mild enlargement of the mean cortical representation area of intact perioral muscles on both hemispheres though it was not significant. We have concluded that there was a cortical reorganization in the hemisphere contralateral to the paralytic side resulting in an increase at corticofugal output related to intact perioral muscles.

Assessment of serum endothelin-1 levels in rat appendicitis model and the effects of bosentan.

OBJECTIVE: In this study, the diagnostic value of serum endothelin-1 (ET-1) levels and the therapeutic effects of bosentan have been investigated in an experimental appendicitis rat model. MATERIALS AND METHODS: Twenty-one male Sprague-Dawley rats were chosen for the study. The rats were allocated into three groups as follows: Group 1 (control, n = 7), Group 2 (appendicitis, n = 7), and Group 3 (bosentan treatment, n = 7). At the 6th hour of the experiment, Groups 1 and 2 received 2 ml saline, and group 3 received 30 mg/kg bosentan intraperitoneally. At the 24th postoperative hour, all rats were sacrificed and evaluated histopathologically to score the severity of appendicitis. The plasma malondialdehyde, reduced and total glutathione levels, serum, and appendiceal tissue ET-1 levels were evaluated. RESULTS: In this study, we found that the ET-1 levels were significantly increased with appendicitis (p = 0.018). The administration of bosentan can statistically significantly both decrease the histopathologic injury in the inflamed appendix and increase the serum total glutathione levels (p = 0.002). CONCLUSIONS: The increase in plasma ET-1 levels may have a diagnostic value of acute appendicitis. We believe that manifestations that occur during the acute phase of appendicitis may be reduced with the administration of bosentan, which may also help prevent complications.

Pedicled Vascularized Lymph Node Transfer Treats Lymphedema in Rat Hind Limb: A Simple Experimental Study Design.

Vascularized lymph node transfer is a new and promising technique for the physiologic treatment of lymphedema and several clinical and experimental studies have been conducted in recent years. However, the exact mechanism of vascularized lymph node transfer is still unknown. In this study, we aimed to investigate treatment efficacy through the design of a simple and feasible experimental lymphedema model for testing a vascularized lymph node transfer technique. After a pilot study, 30 male Sprague-Dawley rats were divided into two groups and lymphedema was induced in the hindlimb of both groups. In Group 1 (control, n=15) no treatment was applied while pedicled lymph node transfer was applied in Group 2 (experimental, n=15). Model dynamics were assessed with lymphoscintigraphy, limb measurement, and histological analysis. A statistically significant limb circumference reduction at the ankle was seen on days 30 and 90 in Group 2 as compared to Group 1, p<0.05, and lymphatic tracer transport improved in 13 out of the 15 animals in Group 2. A statistically significant reduction in histological scores was achieved in Group 2, p<0.05. In this rat hindlimb lymphedema model, our vascularized lymph node transfer technique is an effective physiologic surgical treatment and represents a feasible experimental model for future studies.

Clinical experience with three combination regimens for the treatment of high-risk febrile neutropenia.

INTRODUCTION: The objective of this study was to compare the safety and efficacy of ceftazidime (2 g every 8 h), piperacillin/tazobactam (4 g/500 mg every 6 h), and meropenem (1 g every 8 h), when combined with amikacin (15 mg/kg once daily), in the empirical treatment of high-risk febrile neutropenic episodes in patients with haematological malignancy. MATERIALS AND METHODS: A prospective, comparative study designed in the haematology unit of a university hospital in Turkey. RESULTS: A total of 89 febrile episodes in 60 neutropenic patients were treated; 29 febrile episodes in 23 patients with ceftazidime plus amikacin (group 1), 30 episodes in 25 patients with piperacillin/tazobactam plus amikacin (group 2), and 30 episodes in 25 patients with meropenem plus amikacin (group 3). The 3 groups were comparable in terms of age, sex, underlying malignancy, pretherapy neutrophil counts, duration of neutropenia and types of infections. Neutropenia, since the start of fever, persisted for > or =10 days in all of the episodes in the 3 study groups. Nearly all of the episodes were seen in patients with acute leukaemia. In 25.8% (23/89) of the febrile neutropenia episodes, an aetiologic organism was isolated, with gram-negative bacteria being the most commonly isolated. The success without modification rates were 34.5%, 30% and 36.7% for groups 1, 2 and 3, respectively (P >0.05). After modification with a different class of antimicrobial therapy, the response rates increased to 65.5%, 63.3% and 70% for groups 1, 2 and 3, respectively (P >0.05). The mean duration of treatment and the time to defervescence were also comparable in all groups. In all arms, side effects were minimal. CONCLUSIONS: It is concluded that the 3 regimens were equally effective and safe in the empirical treatment of high-risk febrile neutropenic episodes.

Cutaneous metastasis of endometrial carcinoma with hemorrhagic nodules and papules.

BACKGROUND: Endometrium cancer is the fourth most frequent malignancy in women. However, skin metastasis from endometrium cancer is a very rare entity. CASE: A 58-year-old multiparous woman postmenopausal for ten years presented with multiple metastatic, nodular, hemorrhagic skin lesions located at the initial surgery and radiotherapy site 14 months after the original diagnosis was made. Combination chemotherapy was instituted, but the patient died after the second cycle of chemotherapy. CONCLUSION: Although endometrial cancer is one of the most frequent malignancies in women, skin metastasis from endometrial cancer is very rare. In reported cases metastasis from endometrial cancer has been most commonly noted at the initial surgery and radiotherapy site. Therefore, the initial surgical and radiotherapy site must be examined carefully for skin metastasis.

Oxidant/antioxidant parameters and their relationship with chemotherapy in Hodgkin's lymphoma.

This study investigated changing levels of serum oxidant/antioxidant with chemotherapy and their relation to treatment in 34 Hodgkin's lymphoma patients. The patient population consisted of 19 males and 15 females. Mean age was 30.41 +/- 12.08 years. All patients received the adriamycin, bleomycin, vincristine and dexamethasone (ABVD) treatment protocol. Blood samples were taken before treatment, and on days 1 and 7 during treatment for measurement of superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), catalase (CAT), malondialdehyde (MDA), nitric oxide (NO) and enzyme activities. After ABVD treatment, mean free radical levels were increased and antioxidant levels were significantly decreased in the serum. ABVD treatment results in an increase of free radical levels and a decrease of antioxidant levels in the serum of patients with Hodgkin's lymphoma.

Dysphagia in lateral medullary infarction (Wallenberg's syndrome): an acute disconnection syndrome in premotor neurons related to swallowing activity?

BACKGROUND AND PURPOSE: We have investigated the pathophysiological mechanisms of dysphagia in Wallenberg's syndrome (WS) that are due to lateral medullary infarction (LMI). METHODS: Twenty patients with WS were evaluated by means of clinical and electrophysiological methods that measured the oropharyngeal phase of voluntarily initiated swallowing. For comparison, 22 patients with unilateral hemispheric infarction were investigated during the acute stage of stroke, and 4 patients with unilateral peripheral 9th and 10th cranial nerve palsies were studied. Age-matched 30 healthy control subjects were also included in the study. RESULTS: It was found that dysphagia was clinically more severe in WS patients than in the patients in the other groups. The pharyngeal phase of swallowing was predominantly impaired, whereas in patients with hemispheric stroke, dysphagia was related only to the delay of triggering of the voluntarily induced swallowing. In WS patients, the swallowing reflex was extremely slow in spite of the unilateral involvement due to LMI, whereas the pharyngeal phase of reflex swallowing remained within normal limits in patients with unilateral hemispheric stroke and patients with unilateral peripheral 9th and 10th cranial nerve palsies. CONCLUSIONS: Although in WS the lesion due to LMI is unilateral, its effect on oropharyngeal swallowing is bilateral. In LMI, primarily the premotor neurons in the nucleus ambiguous and their connections seem to be affected. Consequently, a disruption and/or disconnection of their linkage to swallowing-related cranial motor neuron pools bilaterally and to the contralateral nucleus ambiguous could produce the swallowing disorders in WS. However, the remaining intact ipsilateral premotor neurons and the contralateral center in the medulla oblongata may eventually begin to operate and overcome the severity and long-term persistence of dysphagia.

Effect of mucosal anaesthesia on oropharyngeal swallowing.

The effect of the topical anaesthesia of the oropharyngeal mucosae was studied in order to evaluate the role of the mucosal sensory receptors on the oropharyngeal swallowing in 12 adult volunteers. Laryngeal vertical movements were detected by a piezoelectric sensor and electromyography of the submental muscle complex were simultaneously recorded. All subjects were instructed to swallow doses of water, gradually increasing in quantity from 3-20 mL and any recurrence of the signals related to swallowing within 8 s was accepted as a sign of dysphagia and its limit value measured. Before the topical anaesthesia of the oropharyngeal mucosae by xylocaine puffs; the dysphagia limit was never observed with less than 20 mL water. During topical mucosal anaesthesia lasting 4-6 min among the subjects, the dysphagia limit was less than 20 mL water and the recurrence of swallows two or more times was mainly recorded with 3-5 mL water. Five of the subjects demonstrated the clinical and electrophysiological signs of laryngeal aspiration at the earlier period of the topical anaesthesia. It is concluded that the sensory inputs from the mucosal receptors are important to trigger voluntary swallowing and their absence or dysfunction may contribute to oropharyngeal dysphagia and laryngeal aspiration.

A prospective randomized study of alpha-2b interferon plus hydroxyurea or cytarabine for patients with early chronic phase chronic myelogenous leukemia: the International Oncology Study Group CML1 study.

A prospective randomized international study of 143 patients showed no apparent early survival advantage conferred by combining cytarabine, rather than hydroxyurea, with INF as first-line CML therapy. Combinations of alpha-interferon (INF) and chemotherapeutic agents are currently first-line therapy for the majority of patients with chronic myeloid leukemia (CML). The International Oncology Study Group conducted a prospective randomized study comparing INF combined with hydroxyurea or cytarabine. The primary study aim was to compare the survival durations in these patient cohorts. Patients with early chronic phase CML were randomized to receive INF 5 million units (Mu) given five times per week subcutaneously plus hydroxyurea or cytarabine as required to achieve a complete hematologic response and to maintain a WBC count between 2x10(9)/L and 10x10(9)/L and a platelet count between 75x10(9)/L and 100x10(9)/L. Therapy continued as tolerated unless progressive or blast phase disease occurred. At 36 months, the actuarial survival rate was equivalent in both groups: HI group (79 patients) survival was 85% (95% CI, 68-100%), as compared to 95% (95% CI, 79-100%) in the CI group (64 patients). In conclusion if seems that there is no apparent early survival advantage conferred by combining cytarabine, rather than hydroxyurea, with INF as first-line CML therapy.

A prospective randomized study of Chop versus Chop plus alpha-2B interferon in patients with intermediate and high grade non-Hodgkin's lymphoma: the International Oncology Study Group NHL1 Study .

UNLABELLED: The addition of a brief alpha interferon regimen to each CHOP induction cycle, plus one year of alpha interferon thrice weekly maintenance therapy, has no early effect on response rates or survival in patients with Intermediate or High grade cell NHL. BACKGROUND: The CHOP (Cyclophosphamide, Adriamycin. Vincristine, Prednisone) regimen is the most widely used first-line therapy for patients with Intermediate or High Grade (IG/HG) non-Hodgkin's lymphoma (NHL). Alpha 2b interferon (INF) enhances response rates and improves survival in low-grade NHL. The International Oncology Study Group (IOSG) conducted a prospective randomized study comparing CHOP alone or combined with INF in patients with IG/HG-NHL. The primary study aim was to compare the objective response rates in these patient cohorts. PATIENTS AND METHODS: Patients with a confirmed diagnosis of measurable NHL of International Working Formulation (IWF) groups D to H histology were randomized to receive CHOP alone or CHOP with 5Mu INF s.c. for 5 days on days 22 to 26 of each 28 day cycle with INF 5 million units (Mu) given three times per week subcutaneously for 52 weeks in those patients who responded to CHOP plus INF. RESULTS: The overall response rates were equivalent in both groups: CHOP alone (214 patients) 81% (complete 55%, partial 26%); CHOP plus INF (221 patients) 80% (complete 54%, partial 26%). At 36 months, the actuarial survival rate was equivalent in both groups. CONCLUSIONS: There is no apparent early advantage in terms of response or survival conferred by adding the study INF regimen to CHOP therapy for patients with IG/HG-NHL.

Changes in the perioral muscle responses to cortical TMS induced by decrease of sensory input and electrical stimulation to lower facial region.

OBJECTIVE: To determine the changes in the motor cortex due to repetitive electrical stimulation and cutaneous anesthesia in lower facial region. METHODS: A total of 11 subjects participated in the study of repetitive electrical stimulation, and 10 other subjects in the study of lower facial anesthesia. Facial nerve root and face associated cortical MEPs by transcranial magnetic stimulation (eight-shaped coil) were recorded from perioral muscles pre- and post- electrical stimulation and lower facial anesthesia. Cheek near to the corner of the mouth was transcutaneously stimulated by bipolar surface electrode giving repetitive electrical shocks at 5 Hz. Five percent lidocain/prilocain local anesthetic cream was applied to left or right lip-cheek region. RESULTS: There was no significant change in perioral MEP responses after 10-30 min of 5 Hz electrical stimulation. We found a significant increase of amplitude in cortical MEP recordings during lower facial anesthesia especially in cases of cortical magnetic stimulations ipsilateral and contralateral to the anaesthetized side and in perioral recordings contralateral to the anaesthetized side. CONCLUSIONS: The present study demonstrates that topical anesthesia to the lower facial region leads to cortical modulation and fast plastic changes in both hemispheres that are directed to the normal side.

Cricopharyngeal sphincter muscle responses to transcranial magnetic stimulation in normal subjects and in patients with dysphagia.

OBJECTIVE: Cricopharyngeal (CP) muscle of the upper oesophageal sphincter (UES) has a significant role in the pharyngo-esophageal phase of deglutition. The linkage between the CP muscle of UES and the motor cortex has not been previously studied electrophysiologically in healthy humans and in patients with neurogenic dysphagia. METHODS: Needle recordings of EMG responses were carried out from the CP sphincter muscle following transcranial magnetic stimulation (TMS) over the vertex around the Cz electrode position (cortical MEP), and on the parieto-occipital skull and the occiput ipsilaterally (peripheral MEP) in 14 healthy control subjects and in 26 patients with and without neurogenic dysphagia. Needle recordings obtained from the cricothyroid muscle of the larynx were also evaluated in six healthy subjects. RESULTS: The cortical motor latency of CP sphincter muscle was 10.7+/-0.5 ms with an amplitude of 0.8+/-0.2 mV in healthy subjects. Both the latency and amplitude of CP-MEP were facilitated during swallowing. The peripheral MEP of the CP muscle was very stable in all normal subjects (5.1+/-0.3 ms; 1.3+/-0.3 mV) and swallowing did not influence these parameters. The cortically elicited CP-MEP was significantly longer than the cortical MEPs obtained from the cricothyroid muscle of the larynx. In 10 dysphagic patients with corticobulbar tract involvement (6 ALS and 4 pseudobulbar palsy) and with pathologic and hyperreflexic EMG of the CP-sphincter muscle, the cortical MEP of CP muscle of the upper esophageal sphincter could not be elicited, although the peripheral CP-MEPs were obtained. TMS never produced a swallowing movement in neither healthy subjects nor patients. CONCLUSION: The CP muscle of the upper esophageal sphincter can produce MEPs by cortical TMS and by stimulation at the root/nerve levels of vagus nerve. The MEP latency values and central motor delay suggest that there is an oligosynaptic corticobulbar pathway to the motoneurons of CP muscles. When the pathway is affected by a pathology (i.e. ALS or pseudobulbar palsy) the CP sphincter becomes hyperreflexic due to disinhibition and the cortical MEP of the CP muscle disappears due to degeneration of the corticobulbar pathway. These mechanisms appear to be responsible for the pathogenesis of dysphagia.

Bilateral hearing loss during vincristine therapy: a case report.

Vincristine sulfate is a chemotherapeutic agent used in different cancer therapies. It is also the first choice of treatment for peripheral T-cell lymphoma with cyclophosphamide and adriamycin. Sudden hearing loss during vincristine therapy is a very rare event. This is a case of a 16-year old girl who developed sudden bilateral hearing loss related to vincristine therapy.

Bilateral transient hearing loss associated with vincristine therapy: case report.

Vincristine, adriamycin, and dexamethasone (VAD) chemotherapy protocol is first-choice treatment in newly diagnosed multiple myeloma patients in many centers. Sudden hearing loss associated with vincristine therapy is a rarely observed event in the VAD protocol. We describe a 69-year old male patient diagnosed with multiple myeloma 7 months ago who developed sudden bilateral hearing loss related to vincristine therapy. This uncommon adverse effect of vincristine is discussed and the literature reviewed.

Paroxysmal Nocturnal Haemoglobinuria in the Differential Diagnosis of Unresponsive Iron Deficiency Anemia: A Case Report.

A 16-year-old male patient who was on oral iron treatment for iron deficiency anemia for the last one year was seen at the Haematology clinic with complaints of weakness, pallor, and jaundice. A complete blood count revealed Hb of 4.2 mmol/L, Hct of 0.14, and MCV of 76 fl. A blood smear showed 50% neutrophils, 40% lymphocytes, and 10% monocytes with anisocytosis, poikilocytosis, polichromasia in erythrocytes and normoblasts. Reticulocyte count was under 1%. There was a slight erythroid hyperplasia in the bone marrow aspiration. Biochemical examinations showed total bilirubin of 3.9 mg/dL, indirect bilirubin of 3.4 mg/dL, and lactate dehydrogenase (LDH) of 6085 U/L (220-450). In re-evaluating the history of the patient, he was seen to be complaining of dark discoloration of morning urine. Perl's reaction was found to be positive for hemosiderin in the urine sediment. Because Ham's test was positive, the levels of CD55, 58, and 59 proteins on erythrocyte membranes were found to be lower. The patient was started 32 mg of methylprednisolone and his anaemia was improved by the 14th day of treatment. When evaluating iron deficiency anemia resistant to iron supplementation, PNH should be kept in mind.