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Monday, 30 September 2019

CUERPOS DE PAPPENHEIMER EN MORFOLOGÍA DE SANGRE PERIFÉRICA

Written by Luiza Tofan | Carlos Navarro Morante | Óscar Fuster Lluch , Posted in Volumen11

Figure 1. Pappenheimer bodies in erythroblast and in hemocyte (Blood smear. May-Grünwald Giemsa stain).
We present an image of a peripheral blood smear, stained with May-Grünwald Giemsa (Figure 1). This picture shows erythrocytes with multiple inclusions of bluish, small and irregular granules that occupy a portion of the red blood cell. The inclusions described are also observed in erythroblasts, adopting a perinuclear disposition. These granules, suggestive of Pappenheimer bodies, are morphologically different from other visible inclusions with the same type of staining (Howell-Jolly and basophilic stippling). This corresponds to intracellular iron deposits which were evidenced later by Perls staining (Figure 2).
The presence of Pappenheimer bodies is not pathognomonic of any specific disease; nevertheless, it can help to the etiological diagnosis of: 
  • Congenital sideroblastic anemia or refractory anemia with ring sideroblasts, a disorder classified as myelodysplastic syndrome.
  • Splenectomy.
  • Thalassemias.
  • Drepanocytosis.
  • Toxic (Saturnism, Isoniazide).
The presented peripheral blood smear image belongs to an 84-year-old female patient who was admitted to the Emergency Department after being detected with critical hemoglobin value in a routine laboratory test. Once in the Emergency Department, patient showed deterioration of clinical condition, no fever, hypotension without tachycardia and generalized pallor without signs of active bleeding. The patient also presented a lesion in the sacrum with a greenish-brown and malodorous suppuration.

Initial screening tests showed the presence of severe macrocytic aregenerative anemia (Hemoglobin: 4.5 g / dL, MCV: 102 fL and reticulocytes: 5.55%, but with a reticulocyte production index of 0.8), without other cytopenias. The peripheral blood smear showed intense anisopoikylocytosis of the red blood cells, erythroblasts presence, basophilic stippling and Pappenheimer bodies.

According to these findings, the iron profile was added by the laboratory revealing ferric sequestration with elevated ferritin levels (1291 ng /mL), normal serum iron levels and decreased reticulocytic hemoglobin (19.7 pg) which evidenced an inadequate use and availability of it. Other blood biochemistry tests showed acute renal failure (GFR CKD-EPI: 30 mL/min), important inflammatory syndrome (CRP: 220.3 mg /L) and slightly elevated procalcitonin (0.54 ng /mL) with, 3.4 mmol /L lactate levels in the peripheral venous gasometry. These results lead the diagnosis towards a septic process with probable cutaneous focus and a severe multifactorial anemia with transfusion requirements.

The patient was transferred to the Internal Medicine Department, showing clinical improvement, decrease of the acute phase reactants, as well as partial recovery of renal function after the broad spectrum antibiotic treatment.

However, iron overload was maintained after the resolution of the infectious symptoms. This finding, along with the partial response to the blood transfusion and the dysplastic features described in the peripheral blood smear made us complete the study with a Perls stain. The smear revision confirmed the presence of Pappenheimer bodies with an annular arrangement.

Clinical presentation and analytical findings leaded the diagnosis to a possible myelodysplastic syndrome; therefore a consultation to the hematology department was carried out. Due to the weakness of the patient, the multifactorial etiology of the anemia and the decision to limit the therapeutic effort, it was decided to maintain hospital-based home health care, followed by torpid evolution and patient´s death.

Figura2Figure 2. Pappenheimer bodies in erythroblast (Blood smear. Perls stain).
References

1.    Ford J. Red blood cell morphology. Int J Lab Hematol. 2013;35(3):351-7.
2.    Bain BJ. Diagnosis from the blood smear. N Engl J Med. 2005 4;353(5):498-507.
3.    Merino A. Educación continuada en el laboratorio clínico. 2014-2015. Ed Cont Lab Clín; 20: 41-64.

 

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